Acinic cell carcinoma (ACC) is a malignant epithelial salivary gland neoplasm that most frequently occurs in the salivary glands. However, primary ACC of the lung is extremely rare. Due to its low incidence and the lack of related literature, clinicians lack relevant knowledge. It is prone to misdiagnosis, which delays the treatment of patients. Thus, we report a case of primary ACC of the lung confirmed via surgery and pathology, together with a review of relevant literature, in order to summarize the CT imaging manifestations and the value of CT in the diagnosis and treatment of this disease. The aim of this case report was to improve the understanding of primary ACC of the lung and to improve the diagnostic abilities.