We report on successful adjuvant tamoxifen therapy for a metastasizing sweat gland adenocarcinoma of the scalp in a 64-year-old woman. Before the antihormonal therapy, the patient had undergone repeated surgery for ipsilateral intraparotid, soft tissue, and lymph node metastases and had had disease-free intervals of less than 5 months. As the immunohistochemical analysis of the tumor tissue revealed a 100% nuclear reactivity to estrogen and progesterone receptors, we started empirical tamoxifen citrate therapy, which dramatically changed the course of the disease. The patient has been in complete remission for 3 years. This is the third report in the literature of substantial therapeutic benefit of antiestrogen therapy in metastasizing eccrine gland adenocarcinoma with positive hormone receptor immunohistochemistry. We suggest examining the hormone receptor expression in these neoplasms regularly. A prospective study should be commenced to assess the benefit of adjuvant antihormonal therapy in eccrine gland adenocarcinomas.