RESUMO
Primary cutaneous mucormycosis is very unusual and rarely reported in the literature. The diagnosis is difficult and may mimic several infectious and immunologic diseases. We report a case of vulvar mucormycosis in a pediatric patient with end stage renal disease. A female on her first decade of life presented with a small scar on the right labia majora that in matters of weeks progressed aggressively, ulcerated, extended to the contralateral labia and invaded the entir vulvar region. Subsequent surgical debridement was undertaken. Pathology revealed Mucor species with progressive tissue necrosis. The patient was successfully trea ed with systemic antifungal, wide debridement of the affected area, hyperbaric oxygen therapy and surgical reconstruction of the area