Neonatal extremity gangrene is rare, even rarer are those born with evidence of intrauterine vascular occlusion. Intrauterine limb ischemia has been attributed to several etiological factors which include thromboembolic disease occluding the arteries of the affected limb or compression of the limb during intrauterine life. In this report, we present a case of brachioradial arterial thrombosis associated with mild homocysteinemia and double heterozygosity of methylenetetrahydrofolate reductase 677C-T and factor V Leiden gene mutations. We suggest investigating the neonates and their mothers for possible genetic prothrombotic risk factors when they present with intrauterine thrombosis as this issue is important for management and counseling.

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