Idiopathic nodular glomerulosclerosis (ING) is a rare kidney disease which has a low morbidity with no specific manifestations. It resembles nodular diabetic glomerulosclerosis in pathology features but occurs in nondiabetic patients. The diagnosis and differential diagnosis mainly rely on the immunofluorescence and electron microscopic (EM) findings. Unfortunately, controversies still exist on the exact mechanism and etiology of ING. This article mainly discussed the risk factors, diagnosis and treatment of ING based on the current studies worldwide.