Primary cutaneus apocrine carcinoma is a rare malignancy with about 50 cases reported in the literature. Axilla is the most common site of occurrence, but locations like scalp, anogenital region, ear canal, chest, wrist, finger and eyelid have been described. The neoplasm presents itself as an asymptomatic, slow-growing, solid or cystic mass that varies in color. Most patients have a history of a long-standing neoplasm before the diagnosis is made. The disease is considered to have an indolent clinical course with favorable outcome although more than half of reported patients had regional lymph node metastases at the time of diagnosis. Systemic dissemination to lung, bones, liver and brain is extremely rare with only 14 cases documented in the literature. Wide surgical excision with lymph node dissection upon confirmation of the lymph node metastases remains the only curable treatment. Care and management of the disseminated disease is still challenging. We report a case of a 65-year-old woman with a very aggressive apocrine carcinoma of the scalp prone to local recurrence and distant metastases to lung and bones.