Sunitinib-induced thrombotic microangiopathy (TMA) is a secondary TMA caused by sunitinib. Despite the extensive use of sunitinib in patients with renal cell carcinoma and other malignancies, little is known about this complication of sunitinib. No clinical trials of sunitinib have studied this complication in any group of patients. We here review the normal physiology of vascular endothelial growth factor in the kidney, and discuss the pathogenesis, clinical and laboratory manifestations, pathological changes in the kidney, and the management of this uncommon complication of sunitinib.