Spindle-cell lung carcinoma is a very rare disease, one of the forms of biphasic squamous-cell carcinoma, which can be detected in isolated cases. Despite different descriptions of this pathology, currently there is no consensus about methods of its exact morphological diagnosis and effective treatment. Therefore, it is very important to study all existing at the moment results of spindle-cell carcinoma research for the purpose of systematization and analysis of available data and development of effective methods of the disease control. The review contains modern understanding of clinical medicine about the tumor and different methods of its classification and identification based on worldwide scientific experience. The results of the research of spindle-cell lung carcinoma development causes are discussed, and statistical measures of its detection and used treatment methods are described. Spindle-cell lung carcinoma, frequently affecting male smokers as a large peripheral focus, has unfavourable prognosis even in those cases when the diagnosis was made at the early stage and radical surgery was performed. Distant metastases occur more frequently and earlier than in other tumors of the same group, and survival after recurrence is very low. One of the most promising methods of treatment in case of such malignancy is targeted immunotherapy such as anti-PD-1/PD-L1 antibodies aimed at inhibiting immune control point.