Vulvar carcinoma is the fourth most frequently encountered malignancy of the female reproductive tract. Among vulvar neoplasms, 0.1% to 5% are of Bartholin gland origin. Primary adenoid cystic carcinoma of the Bartholin gland is very rare. To date, only about 60 cases have been reported in the world literature. Microscopic examination reveals a neoplasm of cribriform pattern composed of nests and columns of cells of bland appearance arranged concentrically around glandlike spaces filled with eosinophilic periodic acid-Schiff-positive diastase-resistant material. Immunohistochemically, the tumor cells express low-molecular-weight keratins, carcinoembryonic antigen, lysozyme, alpha1-antichymotrypsin, S100, and type IV collagen. Adenoid cystic carcinoma of the Bartholin gland is a slow-growing but locally very aggressive neoplasm with high capacity for recurrence. Perineural and lymphatic invasion is characteristic of this tumor and may explain its propensity for causing pain and recurrence. The treatment may range from simple local excision to radical vulvectomy, with or without partial to complete regional lymphadenectomy.