We present 21 studies of cases of lung cancer in patients with situs inversus totalis (SIT) published worldwide. The first case was described in 1952. Thirteen patients were from Japan, 4 from Eastern Europe, including 2 Polish cases from the authors` center (Department of Thoracic Surgery, Pomeranian Medical University in Szczecin, Poland), 2 from Western Asia, 1 from the U.S. and 1 from Australia. Male patients (20/21) as well as left-sided lung cancer cases (14/21) and squamous cell carcinoma cases (8/21) dominated in the entire group. Thirteen patients underwent surgical treatment. There were 10 left-sided and 3 right-sided surgical interventions with uneventful intra- and postoperative course. Explorative thoracotomy was performed in one case only on the right side. Upper lobectomy was performed in 5 cases, pneumonectomy in 3 cases, lower bilobectomy and middle lobectomy in one case and lower lobectomy in two cases. Surgery was performed through thoracotomy in 10 cases, VATS-assisted approach in two cases and sternotomy in one case. Descriptions of the surgical anatomy confirmed mirror image of the anatomy in all cases and were consistent with the preoperative CT images. Preoperative diagnosis was discussed including the role of 3-D reconstruction of CT for improving perioperative safety in this group of patients. In conclusion, lung cancer/SIT cases despite inversed but regular anatomy can be operated on radically as cases with normal anatomy with preservation of intraoperative security level.