One of two patients with systemic metastases from a poorly differentiated eccrine adenocarcinoma of the scalp was found to have a tumor positive for estradiol receptors. In the receptor positive patient, after tamoxifen therapy, the lymph node metastasis regressed completely and was associated with full relief of pain from osseous metastases for nearly 3 years. Subsequently, progressive painful osseous metastases in the spine, skull, pelvis, and femur were palliated for shorter periods with sequential systemic therapy with megestrol acetate and fluoxymesterone. Osseous metastases were also palliated with external radiation therapy. In contrast, despite external radiation therapy, brain metastases proved fatal. Tamoxifen was ineffective in the estradiol receptor negative patient. Based on this report, it may be valuable to determine the presence of estradiol receptor protein in eccrine carcinoma as a predictor of response to hormonal therapy.