We reported a case of pseudocoarctation of the aorta associated with severe aortic regurgitation. A 63-year-old male admitted with congestive heart failure due to aortic regurgitation. The blood pressure was 170/60 mmHg in the right arm and the systolic pressure was 132 mmHg in the right leg. To and fro murmur and diastolic grade IV/VI blowing murmur was heard along the lower left sternal border. Aortogram demonstrated marked buckling of the descending aorta at the origin of the left subclavian artery without collateral circulation and bicuspid aortic valve with severe aortic regurgitation. His pressure gradient across the pseudocoarctation was 40 mmHg in the preoperative catheterization. We performed an aortic valve replacement (AVR) without further surgical treatment. The pressure gradient was reduced to 10 mmHg and his symptom improved after AVR. These results suggested that pseudocoarctation of the aorta may require no surgical treatment in some case associated with aortic valve regurgitation because of improvement of high pressure gradient across it by AVR alone.