A 36-year-old female presented to the dermatology service with a pinkish to erythematous ulcerated nodule measuring about 1 cm in diameter, located on the labia majora. The lesion has been slowly increasing in size. Physical examination found features compatible with the diagnosis of neurofibromatosis type 1. Excisional biopsy demonstrated the histological picture of syringocystadenoma papilliferum. The interest of this report lies in the rarity of syringocystadenoma papilliferum, its unusual presentation in the vulva, and it's association with neurofibromatosis type 1. To our knowledge, this association has not been described in the medical literature.