Malignant clear cell hidradenoma is an uncommon tumor of eccrine sweat gland origin. In contrast to the benign form, malignant clear cell hidradenoma tends to invade the surrounding tissue and has a high incidence of distant metastases. For these reasons a wide surgical excision associated to a locoregional lymph node dissection should be performed alter the initial diagnosis. The case of a 17 year old female with this rare lesion is reported; the literature is reviewed and surgical treatment discussed.