Objective To discuss the clinical features and treatment of sinonasal low-grade non-intestinal-type adenocarcinoma (LG non-ITAC).Methods Clinical, imaging, pathological and prognostic data of 4 sinonasal LG non-ITAC patients, who were hospitalized in the Affiliated Hospital of Qingdao University from 2013 to 2022, were retrospectively analyzed.Results The 4 patients included one male and 3 females with onset age of 33 to 70 years old. A right nasal mass was found in 2 cases, one 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 surgical treatment with postoperative radiotherapy in one of them. Immunohistochemistry revealed that CK7 was positively expressed, CK20, CDX2 and villin were negative. All the three patients had neither recurrence nor metastasis. The one patient without treatment died of complicated middle ear malignancy.Conclusions Sinonasal LG non-ITAC is rare in clinic. With insidious onset, its definite diagnosis depends upon histopathological eaxamination, and surgical treatment is effective.