We report a case of eccrine sweat gland carcinoma arising from the occipital region, which later metastasized to the regional lymph nodes and spread widely to the viscera. After wide local excision of the primary tumor, it recurred locally, and then metastasized presumably via regional lymph nodes. Widespread metastasis to visceral organs occurred after a ten-year latent period from the appearance of the original tumor. Histochemical and enzymatic histochemical examinations of the specimens from the original tumor and metastasized lesions obtained at autopsy disclosed the characteristics of eccrine sweat gland tumor.