| Summary: | Spinal epidermoid cysts are rare benign tumors. The etiology can be acquired or congenital. We present a rare case of an 18-month-old girl presented 4 months ago with spontaneous intergluteal swelling fistulized to the skin and lower limbs weakness. Magnetic resonance imaging of the spine demonstrated an intradural tumor from L3 to L5 levels, isointense on T1, hyperintense on T2-weighted images with contrast enhancement after gadolinium injection without any coexistent spinal dysraphism suggested the diagnosis of the dermal sinus. The patient underwent triple-level laminectomy for biopsy and tumor resection. A pearly white tumor was encountered, with a subsequent biopsy confirming it to be an epidermoid tumor. At 6-month follow-up, the neurologic deficit was improved. Spinal epidermoid cysts are rare tumors that evolve slowly. Complete total removal is the treatment of choice.
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