Intramedullary spinal cord abscess in children is extremely a rare infection of the central nervous system and may resemble spinal cord neoplasms. Since this entity is one of the treatable conditions of paraparesis, clinical awareness of patients at risk is crucial for early diagnosis and intervention. Once the abscess is established; immediate surgical drainage along side adequate antibiotics should be instituted. The predisposing factors in the pediatric population differ from the adult population in prevalence. Congenital dermal sinus is implicated as the leading cause of intramedullary spinal cord abscesses in children. The diagnosis of intramedullary abscess should be considered and aggressively ruled out in any patient with a dermal sinus who presents with symptoms of a partial or complete transverse myelitis. Rapid treatment is essential for satisfactory neurological recovery. Prognosis is often unfavorable, if diagnosis is delayed. In the present paper a broad overview of intramedullary spinal cord abscess in children is provided. Epidemiology, predisposing factors, pathogenesis, clinical presentation, diagnosis, management, and prognosis of pediatric intramedullary spinal cord abscesses are discussed. The reviewed data are organized in such a way to constitute a guide for the reader who seeks practical information about this rare but extremely important issue.