Intramedullary spinal cord abscess (ISCA) in children is extremely a rare infection of the central nervous system (CNS); and probably a devastating neurological condition. Clinical awareness of patients at risk is crucial for early diagnosis and intervention; as this entity is one of the treatable conditions of paraparesis. Association with congenital neuro-ectodermal abnormality in children is frequent. This pathology highlights the importance of complete neurological checks of infants as a part of their routine physical examination and early management of patients with congenital dermal sinus, prophylactic surgical resection of such a congenital anomaly is recommended by most authors to prevent serious infections of the CNS. However, once the abscess is established; immediate surgical drainage along side adequate antibiotics should be instituted. This may guarantee improving neurological outcome. In this communication, the authors present their experience with four cases of ISCA in children treated successfully with surgical drainage, intravenous antibiotics and neuro rehabilitation between 2001 and 2006 and discuss their results. We concluded that early diagnosis and treatment is essential; before a devastating mechanico-vascular insult of the spinal cord is established from rapid formation of the abscess and a swift expansion of the spinal cord within the limited intraspinal space.