Intramedullary spinal abscesses are rare and potentially devastating lesions. The first case of spinal abscess was described in 1830 and fewer than 100 cases have been reported subsequently. Only sixteen previous reports have described an intramedullary abscess of the central nervous system secondary to a dermal sinus. Involvement of the entire cord is thus exceedingly rare. We present a case of a 7-month-old girl who had rapidly progressing quadriplegia with urinary and bowel retention and was found to have an intramedullary abscess as a result of a dermal sinus. Spine MRI shows thick irregular ring enhancement appeared within the broadest area of the spinal cord on the thoracic and lumbar area on mid thoracic level to L3 and high cervical area which extended to medulla. Immediate decompressive surgery and antibiotic treatment were performed. Excellent clinical outcome was obtained with a combination of medical and surgical management. Complete neurological assessment and diagnostic study of all patients with a congenital dermal sinus are very important. Prophylatic surgery is indicated in many cases to prevent dangerous and recurrent infections of the central nervous system.
