Two patients with adenoid cystic carcinoma of Bartholin's gland and a review of the relevant literature are presented. With the inclusion of these two patients, there are now 24 cases reported. Both patients had large vulvar masses with a short clinical history, and several local tumor recurrences within the first 21/2 years after radical vulvectomy. The characteristic cribriform pattern and perineural involvement in addition to vascular invasion were present in the pathological material. No metastases were found in the inguino-femoral lymph nodes removed. Both patients are alive, without evidence of local recurrence but with lung metastases. A chemotherapeutic treatment scheme (adriamycin and cyclophosphamide) is underway but it is too early to evaluate its results.