Adenoid cystic carcinoma of the breast is uncommon and comprises less than 1% of all mammary cancers. Its occurrence in the male breast is very rare. We describe the first case of adenoid cystic carcinoma in a juvenile male breast. The patient is alive and well 2 1/2 yrs following subcutaneous mastectomy. The details of the 4 previously reported cases of the same tumor in male breasts are reviewed.