ObjectiveTo summarize the clinical features of advanced sinonasal adenoid cystic carcinoma(ACC), and to explore the relationship between surgical margin, recurrence or metastasis and prognosis.MethodsThe clinical data, treatment and prognosis of 12 patients with advanced sinonasal ACC were analyzed retrospectively. Among them, all of 6 patients with T3 and 6 patients with T4 were not local lymph node and systemic metastases. All patients underwent surgery as initial treatment, 9 cases combined with postoperative radiotherapy and 4 cases combined with chemotherapy. The treatment of them were various when local recurrence or distant metastasis.ResultsThe positive rate of surgical margin was high (75%, 15/20) in 20 cases of initial and recurrent lesions. And those with positive surgical margins were surviving up to 32 years.During the follow-up period, there were 7 cases of local recurrence (2 cases with distant metastasis) and 1 case of simple distant metastasis. The longest survival of patients with local recurrence and distant metastasis was 32 years.ConclusionAdvanced sinonasal ACC has a high positive surgical margin rate and a high local recurrence rate. However, the patients can still survive with tumor for a long time after local recurrence or distant metastasis, and the patient's survival quality should be fully considered when making decisions on radical surgery and treatment of recurrence or metastasis.