Sweat gland carcinoma is a rare malignant tumor considered to be radio- and chemo-resistant. A case affecting a labium majorum recurred after surgical resection and was accompanied by bone and visceral metastases. After four cycles of combination chemotherapy consisting of doxorubicin, mitomycin C, vincristine and cisplatin, complete remission lasting 16 months and prolonged survival (50 months) were obtained. A review of the literature also is presented.