Abstract: Sinonasal adenoid cystic carcinoma (SNACC) is the second most common sinonasal malignancy, following squamous cell carcinoma. Most patients present with advanced stage disease (T3-T4) at the time of diagnosis, with a propensity for perineural invasion, bone invasion, anterior skull base involvement, and high probability of positive margins. After initial treatment, local recurrence and distant metastasis are common, sometimes occurring years later. Imaging studies are valuable for determining the extent of the tumor, but a pathological biopsy remains essential for a definitive diagnosis. Surgical resection is currently the primary treatment modality. Endoscopic surgery offers advantages in reducing complications and improving quality of life while providing comparable survival outcomes to open surgery. For unresectable cases, radiotherapy combined with or without chemotherapy is recommended. Long-term close follow-up is crucial for comprehensive management of the disease. A multidisciplinary team (MDT) provides the core strategy for the diagnosis and treatment of SNACC. The MDT focuses on precise staging and personalized treatment, aiming to balance tumor control, functional preservation, and quality of life improvement. It represents the optimal current management pathway for SNACC.