Adenoid cystic carcinoma is a rare cancer of the head and neck. Incidence within the large salivary glands is about 5%. ACC is characterized by slow growth with a tendency to proliferation along the peripheral nerves, causing difficulties in radical resection, but treatment procedures mainly involve extensive surgery. The presented case concerns a patient with recurrence of ACC who had multiple operations because of cancer recurrence in the past. The authors discuss the characteristics of the ACC and the method of diagnosis and therapy.