Syringoma of the vulva is a rare disorder. The patient presented here is the seventh case in the American literature; she is the only patient with this condition in a series of 1132 women with vulvar disease. Differential diagnosis includes epidermal cyst, cherry hemangioma, angiokeratoma, comedo, soft fibroma, Fox-Fordyce disease, steatocystoma multiplex, and lymphangioma circumstriptum. Although syringoma is usually an incidental finding, the diagnosis should be considered in patients with pruritus vulvae. In such cases, examination of the skin around the eyelids and malar areas where syringoma occurs more commonly may suggest a diagnosis of vulvar syringoma that can be confirmed by histologic examination. Inspection of the vulva in cases with extragenital involvement may lead to a more frequent diagnosis of vulvar syringoma.