Pediatric nondysraphic intramedullary lipoma is very rare, and only limited cases have been reported.In the present case, we present two infant patients with these pathologies who were surgically treated.Previous literature on twenty patients with these diseases who had undergone surgical treatments was analyzed. Surgical treatment should be considered in most symptomatic patients, and laminoplastic laminotomy and internal debulking of the lipoma under intraoperative neurophysiological monitoring are mostly recommended.