Lung cancer is the most common malignant tumour worldwide. Large-cell neuroendocrine cancer of the lung is its rare form; standard treatment in the early stage involves anatomical resection of the pulmonary parenchyma. In patients with situs inversus, which is a rare congenital condition, the surgical treatment of lung cancer is associated with additional difficulties and an increased risk of cardiovascular complications that should be considered during pre-operative assessment. The report presents the case of a patient with situs inversus totalis who underwent left upper lobectomy due to neuroendocrine lung cancer. The difficulties associated with the procedure are described. Anatomical resection of the pulmonary parenchyma is the optimal surgical treatment for neuroendocrine lung cancer, which can also be applied in patients with situs inversus totalis.