In this paper the authors present a case of malignant clear-cell hidradenoma developed on the dorsum of the hand. This is only the eleventh complete report in the literature, including follow-up, and only the third to include necropsy. It is the first to find both lung and myocardial metastases. The tumor was very invasive and was treated first by wide surgical excision and radical node dissection, and when the tumor recurred, amputation of the arm was done. The world literature was carefully reviewed and compared with this case. Owing to the already known failure of radiotherapy, and since chemotherapy was not successful in our patient, the authors advise early aggressive surgery, including block dissection of the regional nodes, and thereafter a careful follow-up.