Abstract
Brooke-Spiegler syndrome is a rare disorder, characterized by the development of skin adnexal tumors, including cylindromas, trichoepitheliomas, spiradenomas. Although these neoplasms are benign in most patients, a malignant transformation can rarely occur. Furthermore, an occasional association between cutaneous adnexal tumors and basal cell adenoma as well as adenocarcinoma of the parotid gland has been rarely described, with approximately 20 cases reported. We report a case of BSS presenting with a malignant eccrine spiradenocylindroma, in a patient with previous history of parotid basal cell tumor.