The thromboembolic complications of nephrotic syndrome are reasonably common, including spontaneous peripheral venous and/or arterial, pulmonary arterial, and renal venous occlusions. However, in comparison to the relatively high incidence of the venous thromboembolic complications with hypercoagulable status, arterial thromboses have been reported much less and it was only 20 cases in the English literature so far. Furthermore, the most cases were pediatric patients rather than adults. Therefore, this report describes an adult nephrotic cases complicated by superior mesenteric artery thrombosis leading to death via catastrophic hospital course. Also, we reviewed the literature in English regarding cases of arterial thromboses in adult nephrotic patients with special interest to locations of thrombosis, underlying histopathologic types of glomerulopathy, and use of steroids or diuretics before its development.