Abstract: Objective To summarize the clinical features of advanced sinonasal adenoid cystic carcinoma, and to explore the relationship between surgical margin, recurrence or metastasis and prognosis . Methods The clinical data, treatment and prognosis of 12 cases of sinonasal adenoid cystic carcinoma were analyzed retrospectively. Among them, there were 6 cases of both T3 and T4 lesions, all without local lymph node and systemic metastases. All patients underwent surgery as initial treatment, and some with postoperative adjuvant radiotherapy (n=9), or adjuvant chemotherapy (n=4), the treatment were various when local recurrence or distant metastasis. Results Among the 20 surgeries for first and recurrent lesions, the rate of positive surgical margins was high (75%, 15/20), and those with positive surgical margins survived up to 32 years; 7 cases of local recurrence (2 combined with distant metastasis) and 1 case of distant metastasis alone during follow-up, and the longest survival of patients with local recurrence combined with distant metastasis was 32 years. Conclusion Advanced sinonasal adenoid cystic carcinoma has a high positive surgical margin rate and a high local recurrence rate, but it 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.