The objectives of this paper are to report a case of apocrine carcinoma and the discussion of aspects related to its diagnosis, treatment, and prognosis. Carcinomas with apocrine differentiation not related to extramammary Paget's disease, ductal breast carcinoma, Moll's glands adenocarcinoma and ceruminous glands carcinoma are very uncommon tumors. We report a case of a 51-year-old black woman who developed apocrine carcinoma lesions in the head and neck region. Two lesions involved her left parotid gland (first tumor and local recurrence), and other involved her submandibular skin. The microscopic aspects were as follows: infiltrative glandular epithelial neoplasm with moderate cellular and nuclear pleomorphism; neoplasic cells with polygonal or circular shape, large nuclei and eosinophilic and granular cytoplasm. The apical decapitation secretion was viewed in a large number of intra-cystic tumor cells. Moreover, we found areas with comedo-necrosis or PAS positive staining (with or without diastase). Based on cutaneous apocrine carcinoma compatibility of the microscopic aspects, we concluded that the tumor in the submandibular skin was probably the primary neoplasm. The patient was treated by surgical excisions, and no evidence of recurrent or metastatic disease has been seen after a follow-up period of 12 months.