Background Antiphospholipid syndrome (APS) is a condition that manifests as venous or arterial thrombosis, as well as complications of pregnancy. APS affecting primarily the arteries is less common when compared to venous complications. We present a case of arterial occlusion resulting in critical limb ischemia (CLI) in a pediatric patient. Methods A 14-year old boy presented with worsening right lower extremity pain and ulcerative lesions of his foot. Laboratory analysis revealed a diagnosis of APS. This case report and review of the literature expands our understanding of arterial manifestations of APS in the pediatric patient. Results The patient was discovered to have proximal occlusion of the superficial femoral artery (SFA), the distal popliteal artery, the anterior tibial artery at the mid-calf, and the posterior tibial artery at the ankle. He underwent a common femoral artery to above-knee-popliteal artery bypass with reversed greater saphenous graft. Follow up after over one-year demonstrated an ABI of 1.0 and no evidence of stenosis in the bypass graft on duplex ultrasound (DUS). Conclusions APS is a complex syndrome with a variety of clinical presentations. This case highlights arterial manifestations of APS and reviews the expanding literature to guide improved patient outcomes.