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Introduction: Nondysraphic intramedullary lipomas of the spinal cord are rare lesions. They are most commonly seen in the second or third decade of life. Their occurrence in the pediatric age group is even rarer. Case Report: The authors describe a 14-month-old child who presented with delayed motor milestones involving the bilateral lower limbs. The child was unable to sit or stand. MRI revealed a dorsally located intramedullary lipoma extending from C7 to D12. The child underwent C7-L1 laminotomy and gross total excision of the lipoma. Postoperatively, the child improved neurologically and attained normal power in the bilateral lower limbs. Discussion: Very few cases of extensive intramedullary lipomas involving the dorsal cord have previously been reported. Only two cases of pediatric extensive dorsal lipomas have been mentioned to date. The authors describe successful surgical excision of a holodorsal intramedullary lipoma in a 14-month-old child. To the best of the authors' knowledge, this is the first case being reported of a holodorsal intramedullary lipoma in the youngest patient. Copyright © 2012 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]