Peripheral arterial thrombosis is a rare complication of nephrotic syndrome that occurs in conjunction with a hypercoagulable state and results in a high rate of limb loss and death. We report a case of brachial artery thrombosis in a pediatric patient with nephrotic syndrome, antithrombin III deficiency, and antecedent vessel trauma. An extensive review of the literature is presented. We conclude that management of arterial thrombosis requires aggressive replenishment of antithrombin III and adequate heparinization in addition to the traditional surgical management. Moreover, arterial puncture should be avoided because of the high risk of thrombosis in these patients.