The pulmonary sarcomatoid carcinoma (PSC) is extremely rarely lung neoplasm. A woman at the age of 55 with a local advanced pulmonary sarcomatoid carcinoma of the right lung and CT data on bilateral adrenal metastases and three brain metastases were established. Diagnosis is placed after bronchoscopy with biopsy and detailed pathochistological and immunohistochemical analysis. PSC is extremely malignant and with high risk of distant haematogenic metastases. This rare clinical case support the need for strict pathohistological and immunohistochemical analysis, a difficult pathohistological differential diagnosis with other primary malignant lung tumors and the assessment of complex treatment. In order to improve the healing results and survival of patients, timely diagnosis is required at early stage with surgical treatment and subsequent adjuvant chemotherapy and targetеd therapy after genetic analysis of surgery or biopsy tissue material.