since first described by quain in 1847 ( 1 ) , hundreds of abdominal aortic stenosis have been reported with many variations in anatomy . though , the complete absence of infrarenal aorta , seen in presenting case , is extremely rare . this represents the first case of complete segmental occlusion of infrarenal aorta in the old patient . a 52-yr - old woman presented to the clinic with one - year history of bilateral claudication . blood pressures were measured at 130/80 mmhg in the forearms and at 100/70 mmhg in the thighs , with ankle - brachial indexes of 0.77 . serologic tests including c3 , c4 , venereal disease research laboratory test ( vdrl ) , and immunoglobulin g were unremarkable except mild elevated erythrocyte sedimentation rate ( esr ) of 22 mm / hr and low tittered anti - nuclear antibody of 1:80 with speckled pattern . the abdominal aorta was reconstituted by multiple collaterals , such as marginal artery of drummond , the anastomosis between superior mesenteric and inferior mesenteric artery , bilateral epigastric , lumbar , and abdominal superficial arteries . for her knee joint arthritis , she was started with methylprednisolon of 8 mg and non - steroid anti - inflammatory drug . after negative results of pathergy test and human lymphocyte antigen b 51 , we ruled out the behcet 's disease and she tapered the medications to stop successfully . she wanted the antihypertensive drug for mild hypertension and was taken amlodipine at 5 mg daily . one year later , computer tomographic angiography showed patent bypass graft and decreased collateral vessels ( fig . the etiology of abdominal aortic stenosis is poorly understood . the young age of most of the patients suggests that the malformation may be congenital . maycock first proposed an unequal fusion of embryonic aorta as a congenital mechanism ( 3 ) . in first month of fetus , the paired primitive dorsal aortas fuse to form the abdominal aorta . incomplete fusion due to infection or inflammation may lead of kinking of the aorta resulting to permanent constriction . congenital rubella has been suggested for such vascular anomaly ( 4 , 5 ) . in alagille syndrome which is congenital pulmonary artery stenosis transmitted in an autosomal dominant inheritance , deletion of the jagged 1 gene has been suggested to relate with abdominal aortic coarctation ( 6 ) . acquired etiologies were also suggested in fibromuscular dysplasia or takayasu 's arthritis ( 7 , 8) . in our patients , several inflammatory presentations including the knee joint arthritis , recurrent oral ulcers , and elevation of esr have suggested the possibility of behcet 's disease . we ruled out the behcet 's disease after the negative study of pathergy test . considering the complete absence of infrarenal aorta without any inflammatory reaction , in review of cases for abdominal aortic stenosis , coarctation , hypoplasia , and middle aortic syndrome were used diversely . the term " coarctation " implies a congenital anomaly , and " hypoplasia " is to longer segment of coarctation . middle aortic syndrome is the clinico - anotomical entity involving long segmental narrowing of the abdominal aorta and various visceral arteries irrespective of its etiology . we described this case by " hypoplasia " , because 4 cm of infrarenal aorta was absent . presenting symptoms of abdominal aortic hypoplasia are related to the extent of the stenosis , and the collateral system . in case involving renal artery , patients typically present with uncontrolled hypertension . infrarenal aortic hypoplasia can give rise to claudication when the collateral circulation is not sufficient . an abdominal aortogram via angiography , ultrasound , computed tomography , or magnetic resolution imaging can be used to diagnose and to determine the location and extent of disease . in case of renal artery stenosis claudication and aneurysmal dilatation also need surgical correction . in this case , as the subrenal aortic stump was too short , graft was interposed between the subdiaphragmatic thoracic aorta to the iliac bifurcation . careful physical examination for inspection of dilated superficial arteries and auscultation of bruits can provide a clue for rare causes of ileofemoral insufficiency such as abdominal aortic stenosis . when suspected , an angiography is mandatory to diagnose , and surgical intervention is necessary to improve severe claudication .