Objectives To analyze the clinical features and treatment of low-grade non-intestinal-type sinonasal adenocarcinoma (LG non-ITAC)of the nasal cavity and paranasal sinuses. Methods Clinical, imaging,pathological and prognosis data of 4 patients, who were hospitalized in the Affiliated Hospital of Qingdao University, from 2013 to 2022 with histopathologically proved LG non-ITAC in the nasal cavity and paranasal sinuses were reviewed. Results Among the 4 patients, 1 male and 3 female, the age of onset was 33~70 years old.Two cases found a right nasal mass, one case had right nasal congestion and runny nose for more than 1 year, and one case had left facial pain for more than 4 months.Three patients underwent surgery treatment and one received postoperative radiotherapy.Immunohistochemistry: CK7 positive expression, CK20, CDX2 and villin were negative.Three surgical patients had no recurrence or metastasis.One case died without treatment and died of complicated middle ear malignancy. Conclusions LG non-ITAC of the nasal cavity and paranasal sinuses in clinical is rare, with insidious onset, definite histopathological diagnosis is needed, and surgical treatment is effective.