Complete situs inversus is a rare congenital anomaly characterized by transposition of organs. We report a case of renal transplantation using a kidney from a living complete situs inversus donor. The recipient was a 59-year-old female with end-stage renal disease because of type 2 diabetes mellitus. The donor was the 56-year-old sister of the recipient with complete situs inversus. CT angiogram of the abdomen and pelvis showed complete situs inversus and an otherwise normal appearance of the bilateral kidneys with patent bilateral single renal arteries and longer renal vein in the right kidney. The patient was taken to the operating room for a hand-assisted laparoscopic right donor nephrectomy. The patient tolerated the procedure well and was discharged home in good condition on postoperative day 1. The recipient experienced no episodes of acute rejection or infection, with serum creatinine levels of 0.8-1.2 mg/dL. Laparoscopic donor nephrectomy in a patient with complete situs inversus remains a technically feasible operation and the presence of situs inversus should not preclude consideration for living kidney donation.