In this retrospective study we give a clinical review of our experience with adenoid cystic carcinoma (ACC) and compare our results with those reported in the literature. Between 1981 and 2000, 74 patients with this disease were treated at our department. Complete resection was reached in 45 cases. Fourteen patients received postoperative radiation. Local control rates at 5, 10 and 15 years were 64%, 56% and 52% with a mean local control time of 11.1 years. Tumour size (P<or= 0.001), perineural invasion (P<or= 0.001) and margin status (P<or= 0.001) showed significant influence on local control. Statistically, we were not able to demonstrate a positive effect for postoperative radiation. Distant control rates at 5, 10 and 15 years were 72%, 62% and 59% with a mean distant control time of 14.4 years. Distant failure was diagnosed in lungs and bones and occurred independently of local treatment outcome. Overall survival rates at 5, 10 and 15 years were 71%, 54% and 37% with a mean overall survival of 11.2 years. Recurrence-free survival rates at 5, 10 and 15 years were 57%, 45% and 37% with a mean recurrence-free survival of 9.1 years. The survival rates at 5, 10 and 15 years after diagnosis of tumour progression were 35%, 15% and 0% with a mean survival time of 4.7 years. Our results provide insights to the unusual nature of ACC, which may be useful for making clinical decisions. However, prospective randomized multicentric studies are needed to define the optimal treatment for ACC with special regard to adjuvant treatment modalities.