We report a case of a 52-year-old man with situs inversus totalis who underwent repair of tetralogy of Fallot in his childhood. He also experienced cerebral embolism at the age of 50 years, and recovered with no sequela after endovascular thrombectomy. Two years later, he was admitted to our hospital due to progressing orthopnea. He was diagnosed with congestive heart failure caused by severe aortic regurgitation with moderate tricuspid regurgitation and pulmonary hypertension. Three-dimensional computed tomography also showed aortic root dilation, single coronary artery, and inverse persistent left superior vena cava (PLSVC) draining into the left atrium, which was assumed to be associated with the previous stroke. A Bentall operation, tricuspid annuloplasty, and concomitant ligation of the PLSVC were performed.