Abstract
Nodular forms of mastocytosis are rather rare skin diseases, especially when localized on the vulva. A 9-year-old girl presented with urticaria pigmentosa type lesions since her 4th year, associated with several solitary or confluent vulvar nodules, varying in size from a pea to a walnut, and mild systemic symptoms. Diagnosis of mastocytosis was confirmed by histology, and apart from splenomegaly, no signs of systemic spread or associated hematologic disorders were detected. Therapeutic response of nodular lesions was rather poor, and further follow up is necessary.