Objective To analyze the clinical, pathological and immunological characteristics of primary non-intestinal adenocarcinoma (non-ITAC) of the nasal cavity and paranasal sinuses with clinical analysis and literature review, so as to provide reference for its diagnosis and treatment. Methods The clinical, imaging and pathological data of 6 cases with non-ITAC of the nasal cavity and paranasal sinuses confirmed by surgery and pathology in our hospital from 2014 to 2020 were analyzed retrospectively. Results Of the 6 cases, 4 were male and 2 were female, with age ranged from 29 to 72 years old (average 54.8 years). Pathology confirmed high grade non-ITAC in 4 patients and low grade in 2. The main clinical manifestations were blood in nasal mucus or sputum and nasal obstruction. All of the 4 males had a history of smoking, and one of them was exposed to sawdust at work. The treatments were surgery and postoperative radiotherapy or chemoradiotherapy. Immunohistochemical results showed that CK7 was positive in 6 cases and P63 was negative in 4, the Ki-67 index was 60% to 90% in the 4 cases with high grade non-ITAC and 1%~2% in the 2 with low grade non-ITAC. One patient died of pulmonary metastasis during follow-up, and none of the other patients had recurrence or metastasis. Conclusion The clinical and imaging features of non-ITAC of the nasal cavity and paranasal sinuses are important for diagnosis, and the pathological immunology is helpful for grading, treatment and prognosis.