We report an unusual case of a 6-year-old boy with a sinus tract terminating with an intramedullary dermoid cyst and holocord central lesion, presenting with tetraparesis secondary to intramedullary abscess. Total excision of dermal sinus tract, dermoid cyst, and the intramedullary abscess by means of a L2-S3 laminectomy, followed by antibiotic therapy resulted in good functional recovery. Strengths of the upper extremities have fully recovered, and a remarkable improvement was detected in the muscles of the lower extremities. Postoperative magnetic resonance imaging (MRI) of the spine showed complete removal of the dermoid cyst, decreased inflamed granulation tissue over the medullary conus, and disappearance of the holocord high intensity lesion. The pathomechanism of holocord central lesion is discussed herein.