Plexiform Neurofibroma of the Vulva in a Patient With Neurofibromatosis Type 1.

Plexiform neurofibroma (PNF) is a rare but pathognomonic tumour associated with neurofibromatosis type 1 (NF-1), an autosomal dominant neurocutaneous disorder. Although PNFs can affect various body regions, vulvar involvement is exceptionally uncommon. This report describes a 16-year-old female patient who presented at a dermatology center with a painless, hanging mass on the right labia majora, accompanied by multiple skin café-au-lait spots, freckling, and cutaneous neurofibromas. Clinical evaluation, genetics testing, imaging, and histopathological analysis confirmed a diagnosis of labia majora PNF. Surgical resection was performed twice due to recurrence. Given the psychosocial and functional challenges, including gait limitation, embarrassment, and psychological distress, associated with this rare presentation, a multidisciplinary approach with an individualised management plan and long-term follow-up is essential. This case emphasises the need to raise awareness of vulvar neurofibromas in NF-1 patients to ensure timely diagnosis and intervention.