A case of pseudo-coarctation of the abdominal aorta associated with renovascular hypertension has been reported. The abdominal aorta of the patient was kinked at the level of L1-L2 without a pressure gradient, which was consistent with pseudo-coarctation of aorta. Both renal arteries arose from the aorta at the level of lower T12 and had severe multiple stenoses. We have discussed the possible etiology of the developmental abnormalities of the arterial system in this patient.