Vascular Ehlers-Danlos Syndrome (VEDS), previously called Ehlers-Danlos syndrome 
type-IV, is a heterogeneous group of heritable connective tissue disorders 
characterized by thin, translucent skin, easy bruising, arterial, intestinal, 
and/or uterine fragility. There is large vessel involvement that leads to 
arterial rupture often preceded by aneurysm, arteriovenous fistulae, or 
dissection. Noninvasive imaging studies such as CT angiography and MR 
angiography are preferred as diagnostic studies for this condition. We are 
reporting a 4 years old girl who was presented with right sided unilateral 
convulsions and hypertension. CT angiogram showed stenosis with post-stenotic 
dilatation of coeliac and superior mesenteric arteries. There were extensive 
calcified plaques with atherosclerotic changes in the segment of right common 
iliac artery with aneurysmal dilatation of celiac, superior mesenteric and 
common iliac artery. Radiological findings were consistent with vascular 
Ehlers-Danlos syndrome. She was successfully managed with anti-hypertensive and 
anticonvulsants.