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Nevus Lipomatosus Cutaneous Superficialis of the Clitoris

BACKGROUND NEVUS LIPOMATOSUS cutaneous superficialis (NLCS) is a relatively rare condition. Although NLCS can affect any region of the entire body, no case of NLCS in the clitoris has ever been reported. OBJECTIVE To report a case of NLCS of the clitoris that presented manifestations that were similar to female pseudohermaphrodism. METHODS This is a case report and review of the literature. RESULTS A congenital, soft, skin-colored lesion of the clitoris in a 1-year-old girl resembled female pseudohermaphrodism. It was resected, and the histologic diagnosis was NLCS. Postoperatively, there was no subsequent change in the lesion during the 3-year follow-up period. CONCLUSION We reported a case of congenital solitary NLCS of the clitoris. Although NLCS might be a relatively rare condition, we should consider it in the differential diagnosis of female pseudohermaphrodism.