A retrospective review of 14 patients with adenoid cystic carcinoma of the tongue treated between 1955 and 1997 was performed. Treatment consisted of surgery (n = 2), radiotherapy (n = 2), chemotherapy (n = 1), or combination therapy (n = 9). The 2-, 5-, and 10-year absolute survival rates were 92%, 79%, and 63%, respectively. Seventy-five percent of the patients who died of cancer succumbed to distant metastases. However, long-term survival was common despite a high incidence of local and distant recurrence. The presence of positive surgical margins, the incidence of regional metastases, the incidence of perineural invasion, the initial stage of disease, and the eventual development of locoregional recurrence and distant metastases did not significantly alter the survival rate. Surgical extirpation combined with postoperative radiotherapy is advocated for the treatment of adenoid cystic carcinoma of the tongue. Given the indolent nature of this disease process, surgery should be directed toward conservation of speech and swallowing function.