A case of an 11-year-old boy with Ehlers-Danlos syndrome (EDS) type IV is p
resented. He was referred with a pulsatile mass above the right antecubital
fossa and Doppler revealed a saccular aneurysm. Excision and ligation of t
he brachial artery aneurysm was performed. The diagnosis was confirmed by s
kin fibroblast cultures and histopathologic evaluation. We conclude that ar
teriography should be avoided in EDS and color-Doppler should be the sole m
eans of diagnosis. Ligation rather than arterial reconstruction should be p
referred.