Carcinoma of sweat glands is a very rare neoplasm of dermal appendages that is difficult to diagnose clinically and histologically. It has a potential for rapid growth and metastasis. Carcinoma of eccrine sweat glands, also known as eccrine carcinoma, is a distinct histologic subtype of sweat gland carcinoma. We report our autopsy findings and experience with chemotherapy in a patient with metastatic eccrine carcinoma. The pertinent literature and histologic classification of eccrine carcinomas are reviewed.