A review of the literature reveals that fewer than two dozen pediatric cases of nondysraphic intramedullary lipoma have been described, with most lesions spanning limited spinal segments. Extensive or holodorsal involvement has been reported only rarely, including isolated cases involving long cervicothoracic segments or multilevel disease. The present case is notable not only for the patient’s age, but also for the exceptional size and extent of the lesion, exceeding that of most previously reported pediatric nondysraphic intramedullary lipomas. This distinction is clinically relevant, as lesion size and longitudinal extent may increase operative complexity and the risk of postoperative neurological deficit and spinal deformity. Because of their rarity, optimal management strategies for nondysraphic intramedullary lipomas remain incompletely defined. Complete resection is often precluded by the absence of a clear dissection plane between the lipoma and normal neural tissue, and subtotal debulking is generally favored to minimize neurological morbidity. Despite these challenges, timely surgical intervention in symptomatic patients can halt neurological deterioration and allow for meaningful functional recovery. We present a case of a nondysraphic intramedullary spinal cord lipoma that, on review of literature, represents one of the largest reported nondysraphic intramedullary lipomas in a pediatric patient. This case adds to the limited body of literature on this rare disease process and underscores important considerations regarding diagnosis, surgical strategy, and long-term management.

Received: 14 April 2026

Accepted after revision: 19 June 2026

Article published online:
23 July 2026

© 2026. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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