Primary adenoid cystic carcinoma of the nasal cavity and paranasal sinuses is rare. The tendency to spread along the perineural space, the high rate of local recurrence, and at least 40% distant metastatic rate, together with a complex anatomic situation, make its prognosis worse than that in any other area of the head and neck region. Between 1971 and 1986, 10 patients with adenoid cystic carcinoma of the nasal cavity and paranasal sinuses were primarily treated at VGH-Taipei. After long-term following-up, there were 9 local recurrences in 5 patients, 4 distant metastases, and 5 patients died of the disease. The local control rate was 33.3% and 2 patients were followed for more than 10 years without recurrence. Anatomically permitted wide surgical excision with adjuvant radiotherapy is the treatment of choice.