Background: Spinal epidural lipomatosis (SEL) is a rare disorder characterized by excessive epidural fat accumulation. Diagnosis may be challenging because imaging findings are frequently overlooked, and evidence-based management guidelines, particularly thoracic disease, remain limited. Case report: We present a case report of a 54-year-old male patient, who suffered from lower limb spastic paresis and associated muscle spasms (Nurick grade V). After a difficult diagnostic process, MRI of the thoracic spine confirmed dorsal/posterior spinal epidural lipomatosis (SEL; Manjila grade IIb-α) extending from Th2 to Th9, with moderate thecal sac compression. A small Th6–Th7 intervertebral disc prolapse was also present, without evidence of scoliosis, syrinx, spinal cord edema, or myelomalacia. Following bilateral thoracic decompression and complete resection of the SEL tissue, the patient’s neurological function improved markedly, from Nurick grade V preoperatively to Nurick grade II at 11 months of follow-up. Consequently, bilateral laminectomy of the thoracic region was performed with total resection of the SEL tissue. Following surgery, the patient’s neurological symptoms improved markedly (Nurick grade II; 11 months postoperatively), and histopathological examination confirmed the diagnosis of spinal epidural lipomatosis. Methods: We performed a systematic review of case reports of SEL occurring, or co-occurring in the thoracic region based on the literature identified on PubMed, Scopus and Web of Science databases, after using search terms: “(thoracic) AND (“spinal” OR “epidural”) AND (lipomatosis)” and filters: [English], [humans] and [year 2020–2026], with the accordance to the PRISMA guidelines. Results: We identified 21 cases of thoracic SEL from 19 articles. In 12 cases the patients were male, in 9 female. Reported etiological associations included type 1 diabetes mellitus, exogenous corticosteroid exposure, obesity/metabolic syndrome, Cushing syndrome, and CLOVES syndrome. Given the small, case-report-based sample, these findings should be considered reported associations rather than estimates of prevalence or independent risk factors. Surgical treatment of thoracic SEL can be accomplished by various techniques such as laminectomy (continuous or skip), laminoplasty, or minimally invasive procedures—including endoscopic spine surgery. Conclusions: Large-volume, multicenter studies with long-term clinico-radiological follow-up would be necessary to understand the natural history and treatment outcomes, and to form evidence-based guidelines for interventions. Our case report and findings highlight that thoracic SEL should be included in the differential diagnosis of unexplained compressive myelopathy, even when imaging studies are inconclusive. Rapid identification or possibly reversible causes of myelopathy and initiation of treatment can significantly relieve symptoms, so it is important to remember this disease in the diagnostic process.