Lipoprotein glomerulopathy is a rare disease characterized by proteinuria, renal function insufficiency, and lipoprotein metabolism dysfunction. The main pathogenesis of the disease is apolipoprotein E (apoE) gene mutation. Lipoprotein glomerulopathy is a rare ridney disease, with its incidence being unknown. This article summarizes the pathophysiology, clinical and pathological manifestations, diagnosis and differential diagnosis of lipoprotein glomerulopathy, as well as the potential effective treatments.