A tortuous thoracoabdominal aorta is a rare congenital abnormality with an unknown prevalence due to rarity. A bicuspid aortic valve is the most common congenital cardiac anomaly and is found in 0.5-2% of the population. The coexistence of these two abnormalities together with severe aortic stenosis is much rarer. We report a 78-year-old male who admitted to the Hospital with a history of dyspnea. Following echocardiography and computed tomography scan, the patient was diagnosed with a bicuspid aortic valve, severe aortic stenosis, and severe thoracoabdominal aortic tortuosity. Transcatheter aortic valve implantation was unsuccessful so he underwent aortic valve replacement. The post-operative course was uneventful.
