Objective: Intramedullary melanocytomas are extremely rare spinal cord tumors with distinct histopathological and imaging characteristics. This report reviews the literature on this pathology and presents a representative case study, highlighting aspects of diagnosis and management. Methods: A scoping review of PubMed, Web of Science, and Embase databases was conducted to identify reports on intramedullary melanocytomas, focusing on clinical presentation, imaging features, histopathology, treatment, and outcomes. Case reports and case series were included due to the rarity of these tumors. Results: Twelve manuscripts met the inclusion criteria, including 15 patients. In the majority of patients, intramedullary melanocytomas present with progressive myelopathy and pain. Most common MRI findings include hyperintensity on T1-weighted images, iso- to hypointensity on T2-weighted images, and homogeneous contrast enhancement. Intralesional cysts and associated syrinx are common. Gross total resection (GTR) remains the primary treatment, but complete removal is often challenging due to tumor adherence to neural structures. Conclusions: Intramedullary melanocytomas require careful diagnosis and management due to their diagnostic overlap with malignant melanoma and potential for recurrence. While GTR is the mainstay of treatment, long-term surveillance is warranted due to high recurrence rates. Further research is needed to define the natural history of the disease and establish optimal therapeutic strategies.