Situs inversus totalis refers to an inverted position of part or all of the viscera, which represents a mirror-image of the normal location, and it is a relatively rare condition that occurs in one in 4,000-8,000 people in Japan. We herein report a patient demonstrating a combination of situs inversus totalis, colon cancer with hepatic metastasis, and cholecystolithiasis, who was treated surgically. We found no expression of UVRAG mRNA in our case, thus suggesting that the UVRAG gene is partly responsible for this condition.