A patient with adenoid cystic carcinoma of the vulva treated primarily with surgery developed multiple local and distant recurrences in which radiotherapy repeatedly achieved complete local control. The patient survived 11.5 years from original diagnosis and 9.5 years from primary recurrence before dying of pulmonary metastases. Adenoid cystic carcinoma of the vulva is a highly radiosensitive tumor. Adjuvant radiotherapy should be considered as a part of the primary therapy, to improve local control, on the basis of the much larger experience with adjuvant radiotherapy with this tumor in the head and neck. Also on the basis of head and neck tumors, it is unlikely that radiotherapy will affect survival.