✓ An intramedullary abscess of the spinal cord (IASC) represents a rare disease associated with a potentially devastating outcome. Few cases involving children suffering from an IASC have been reported in the neurosurgical literature. In the majority of the reported pediatric cases there were either congenital abnormalities, such as a dermal sinus, or signs of local infections leading to a secondary hemopoietic spread. The authors report the case of an 18-month-old girl with an extensive IASC associated with an epidermoid cyst extending from T-11 to S-2 without evidence of a dermal sinus or history of clinically apparent systemic infection. To their knowledge, this is the first case report of an IASC without a condition facilitating either direct contamination via a dermal sinus or hemopoietic spread from an infectious focus outside the central nervous system. Signs and symptoms, the clinical course, and imaging features are discussed and the relevant literature is reviewed.