Situs inversus totalis is a rare anomaly characterized by the total inversion of all abdominal and thoracic organs. For the first time, we report a case of a donor nephrectomy in a patient with situs inversus totalis, completed with a full laparoscopic approach. At the time of this writing, the donor and the recipient are doing well after 6 month's follow-up. Our experience shows that patients with situs inversus totalis may be eligible candidates for laparoscopic donor nephrectomy, provided that detailed preoperative imaging studies as well as precise preoperative planning are performed before the transplant.