Neurofibromatosis 1 is an autosomal dominant disorder with cutaneous findings that include multiple café-au-lait spots, axillary/inguinal freckling, dermal, and plexiform neurofibromas. Skin manifestations, including involvement of the vulva, are often the most troubling physical finding to patients. Hormonal and growth factor changes during puberty have been implicated in neurofibroma growth. In the case presented here, an exceedingly rare isolated vulvar neurofibroma without clitoral involvement became enlarged and symptomatic, requiring excisional surgery after puberty. The diffuse involvement of these tumors makes complete resection very difficult and recurrence is common.
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