A patient had primary cutaneous adenoid cystic carcinoma. The tumor closely resembled adenoid cystic carcinoma of salivary gland origin and was composed of cribriform and tubular masses of basaloid cells. Lumina formed by neoplastic cells contained mucin and hyalin , and similar constituents were located between neoplastic cell masses and adjacent dermis. Perineural invasion was observed. A review of seven other primary cutaneous adenoid cystic carcinomas indicates that this neoplasm tends to affect middle-aged and older individuals, is often present for several years prior to diagnosis, has a propensity for local recurrence, but has not produced metastases in regional lymph nodes or distant sites. Therapy should employ wide surgical excision that extends well beyond the clinical confines of the neoplasm.