We report a rare case of vulvar adenoid cystic carcinoma (ACC) with a squamous cell carcinoma (SCC) component. A 78-year-old woman presented with a nodule in the left side of her vulva for 30 years. In the past few years, the nodule had increased in size and was accompanied by pain and discomfort during squatting. Physical examination revealed a firm vulvar chicken egg-sized mass with poor mobility. The findings of routine blood tests, including major tumor markers, were within normal ranges. No abnormalities were observed in the internal genitals or intestinal tracts. Magnetic resonance imaging revealed a tumor located under the skin of the perineum with a cystic change in fibrous partitions. It showed high intensity on T1-weighted images and high intensity with nonuniformity on T2-weighted images. No metastatic le-