Plexiform neurofibroma presenting as ambiguous genitalia

Abstract

Plexiform neurofibroma rarely affects the external genitalia and is a rare cause of clitoromegaly. The essential aspect of the management is doing a clitoroplasty with the preservation of glandular and neurovascular tissues. We present the case of an 18‑year‑old female who had ambiguous genitalia due to an enlarged clitoris due to neurofibromatosis that was the size of a penis with indistinct labia majora. She was managed by carrying out a feminising genitoplasty, a combination of clitoroplasty and labiaplasty.