The present article describes a 15-year-old girl with two arteriovenous malformations (AVMs) — on her left labia majora and left thigh — in association with genetically confirmed de-novo neurofibromatosis type 2. This patient's labia majora AVM was particularly difficult to treat using embolization techniques. She experienced numerous complications including pulmonary edema, venous thromboemboli, severe local area necrosis and a damaged anal sphincter. She required surgical debridement and reconstruction of her suprapubic and perineal regions. The left thigh AVM was later treated using a combination of embolization and surgical resection with no complications. Six years later, she is doing well. To the authors’ knowledge, the present report is the only case of neurofibromatosis type 2 associated with extracranial arteriovenous malformations described in the literature.

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