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.

Keywords

• clitoral neurofibromatosis
• clitoromegaly
• clitoroplasty
• feminising genitoplasty