Introduction Situs inversus totalis represents a rare congenital anomaly in which the thoracic and abdominal viscera are transposed to the opposite side through the sagittal plane. It often occurs concomitantly with other disorders that present a challenge for making the diagnosis and management of abdominal pathology. The relationship between situs inversus totalis and cancer remains unclear. This study reports a rare case of adenocarcinoma of the gall bladder in a patient with situs inversus totalis. Case report We report a first clinical case of a 50-year-old man with situs inversus totalis with adenocarcinoma of gall bladder, which was successfully treated with surgery and doing well with no evidence of local recurrence or metastasis during six months of follow-up. Conclusion The occurrence of gall bladder adenocarcinoma in a patient with situs inversus totalis accounts as a very rare occurrence, as no case is reported till date. In this context, when the tumour is resectable, surgical management should be considered and must be preceded by a careful preoperative staging and full adaptation to the mirror image anatomy.