Background: Extraspinal sacrococcygeal ependymoma is a rare pediatric neoplasm that frequently presents as a superficial sacrococcygeal mass, closely resembling benign conditions such as pilonidal disease or cysts. As a result, preoperative diagnosis is often incorrect, leading to incomplete surgical excision and delayed clinical management. We conducted a comprehensive descriptive review of the pediatric literature to examine its epidemiology, clinical presentation, diagnostic challenges, pathological characteristics, treatment strategies, and outcomes. Materials and Methods: A structured literature search of PubMed, Scopus, and Web of Science from database inception to June 2026 was conducted. Only English studies reporting histologically confirmed extraspinal sacrococcygeal ependymoma in patients younger than 18 years were included. Discussion: Histologically, the vast majority of extraspinal sacrococcygeal ependymomas are of the myxopapillary subtype. Historically classified as grade 1 tumors by the World Health Organization (WHO), they were reclassified as WHO grade 2 in the 2021 WHO Classification of Tumors of the Central Nervous System. This change reflects the rising recognition of the clinically relevant risk of local recurrence and metastatic dissemination of these tumors, despite a relatively indolent growth pattern. Because extraspinal sacrococcygeal ependymomas may recur many years after initial treatment and occasionally metastasize to lymph nodes, lungs, liver, bone, or other distant sites, they should not be considered entirely benign lesions. Then, their management requires an oncological approach despite their often innocuous clinical appearance. Conclusions: Given the risk of delayed recurrence and distant metastasis, long-term follow-up is mandatory. Increased awareness of this singular entity, appropriate preoperative imaging of atypical sacrococcygeal lesions, and multidisciplinary management are essential to optimize outcomes. Future multicenter collaborative studies are needed to establish evidence-based recommendations for diagnosis, treatment, and surveillance.

Background: Extraspinal sacrococcygeal ependymoma is a rare pediatric neoplasm that frequently presents as a superficial sacrococcygeal mass, closely resembling benign conditions such as pilonidal disease or cysts. As a result, preoperative diagnosis is often uncorrect, leading to incomplete surgical excision and delayed clinical management. We conducted a comprehensive descriptive review of the pediatric literature to examine its epidemiology, clinical presentation, diagnostic challenges, pathological characteristics, treatment strategies and outcomes. Materials and Methods: A systematic search of PubMed, Scopus, and Web of Science from database inception to June 2026 was conducted. Only English studies reporting histologically confirmed extraspinal sacrococcygeal ependymoma in patients younger than 18 years were included. Discussion: Histologically, the vast majority of extraspinal sacrococcygeal ependymomas are of the myxopapillary subtype. Historically classified as grade 1 tumors by World Health Organization (WHO), they were reclassified as WHO grade 2 in the 2021 WHO Classification of Tumors of the Central Nervous System. This change reflects the rising recognition of the clinically relevant risk of local recurrence and metastatic dissemination of these tumors, despite a relatively indolent growth pattern. Because extraspinal sacrococcygeal ependymomas may recur many years after initial treatment and occasionally metastasize to lymph nodes, lungs, liver, bone, or other distant sites, they should not be considered as entirely benign lesions. Then, their management requires an oncological approach despite their often innocuous clinical appearance. Conclusions: Given the risk of delayed recurrence and distant metastasis, long-term follow-up is mandatory. Increased awareness of this singular entity, appropriate preoperative imaging of atypical sacrococcygeal lesions, and multidisciplinary management are essential to optimize outcomes. Future multicenter collaborative studies are needed to establish evidence-based recommendations for diagnosis, treatment, and surveillance.