We report a case of primary adenoid cystic carcinoma of the skin in a 44-year-old Brazilian man that was present for 6 years with no metastasis. It is a rare malignant neoplasm of the eccrine gland. Metastases have been found in a few cases, usually to the lungs or lymph nodes. This neoplasm microscopically presents with large nests of cells with adenoid or cribriform features and many epithelial small solid islands. Histopathologic analysis is necessary for diagnosis.