We report two unusual cases of a 17-month-old boy with a previously undiagnosed lumbar dermal sinus tract terminating in an intradural dermoid cyst and holocord edema or syrinx, presenting with paraparesis and sphincter dysfunction secondary to an intramedullary abscess and a 26-month-old boy with a previously undiagnosed lumbar dermal sinus tract terminating in an infected dermoid cyst and intramedullary abscess, presenting with recurrent episodes of meningitis and hydrocephalus. Pre-operative magnetic resonance imaging (MRI) studies in these patients were initially confused for an intramedullary spinal cord tumor; however, the presence of an associated dermal sinus tract made this diagnosis of neoplasm less likely. Total excision of the dermal sinus tract, debulking of the dermoid cyst and drainage of the intramedullary abscess through an L1-L5 osteoplastic laminoplasty and midline myelotomy, followed by long-term antibiotic therapy resulted in a good functional recovery. Post-operative MRI of the spine showed removal of the dermoid cyst, decreased inflammatory granulation tissue and resolution of the holocord edema or syrinx. We also performed a literature review to determine the cumulative experience of management of intramedullary abscess in this rare clinical setting.