Clear cell hidradenocarcinoma is a malignant tumor of the sweat glands that represents less than 0.001% of the skin appendages malignant tumors. The original description is attributed to Keasbey and Headley in 1954. Its origin can be primary or it can be developed on a pre-existing benign neoplasm [1][2][3]. This neoplasm mainly affects male patients with an average age of 50. The most frequent topographic location is on the head followed by the neck and extremities [3]. In most of the cases, it is presented as a solitary tumor of a variable size of 1 to 5 cm, not well demarcated, either ulcerated or not. Typically, asymptomatic, slow growing and developing metastases approximately 60% within the first 2 years, the 5-year survival rate, is less than 30% [3]. The diagnosis is confirmed by a histopathologic study. The treatment of choice is a surgical procedure with wide margins. Other alternatives are chemotherapy and radiotherapy [3].