Objective To explore the clinical and CT features of SMARCA4-deficient non-small cell lung cancer (SMARCA4-dNSCLC ). Methods SMARCA4-dNSCLC cases confirmed histopathologically were enrolled and analyzed retrospectively in our hospital from January 2018 to January 2022. Using SMARCA4-intact NSCLC (SMARCA4-iNSCLC) cases admitted during the same period as control, the clinical characteristics and CT features of SMARCA4-dNSCLC cases were observed, recorded and compared. Results There were 42 cases in the SMARCA4-dNSCLC group and 43 cases in SMARCA4-iNSCLC control group, respectively. Compared with the control, the SMARCA4-dNSCLC group had a higher proportion of males, smokers, and chronic obstructive pulmonary disease (COPD). Clinically, the SMARCA4-dNSCLC group exhibited a higher incidence of superior vena cava syndrome, while the SMARCA4-iNSCLC group presented with hemoptysis more frequently. On CT imaging, tumors in the SMARCA4-dNSCLC group showed relatively homogeneous density, fewer cystic changes, necrosis, or calcification, more ill-defined borders, and a lower rate of associated obstructive pneumonia/atelectasis. Conclusions SMARCA4-dNSCLC was more frequently seen in elderly males and heavy smokers and was frequently accompanied by COPD. Occasionally, it could present with superior vena cava syndrome. The tumors were prone to locate in the upper lobes of both lungs. On CT imaging, it mostly presented as a soft tissue mass with relatively homogenous density, ill-defined boundaries, without cystic changes or cavities, and calcification was rare. The vast majority showed significant heterogenous enhancement after contrast. Mediastinal lymph node metastasis was common. This unique subtype of NSCLC should be considered in patients with the above clinical and CT features.