A 13-year-old girl was admitted to the hospital with the decrease in visually acuity. Her eyes examination revealed bilateral lens subluxation subluxation and grade II hypertensive retinopathy. Her blood pressure was 160/110 mmHg and her height was 167cm (90-97th percentiles). Other findings included a large nose, a long facies, a high-arched palate, long fingers and a grade II diastolic murmur at the right upper sternal border. She was diagnosed as having Marfan syndrome. Firstly, the most common causes of hypertension were investigated. Because all screening evaluations were normal, she underwent a digital subtraction angiography (DSA) of aorta and bilateral selective renal angiography for suspected renal artery (RA) stenosis. Digital substraction angiography revealed a prominent kink at right RA and an aneurysm at left RA. The left RA was twisted and tortuous (Fig. 1). © © Copyright 2010 by AVES Yayincilik Ltd.