Lipoprotein glomerulopathy is a rare disorder characterized by proteinuria, renal insufficiency and disturbances in lipoprotein metabolism closely related to those observed in type III hyperlipidemia. Rare mutations in apolipoprotein E (apoE) gene may contribute to the pathogenesis of the disease. This article reviews the clinical and laboratory features of lipoprotein glomerulopathy, discusses the mechanisms that may be implicated in its pathogenesis and summarizes the currently available therapeutic options.During the past years two new apoE gene mutations were described in Caucasian patients, apoE Modena (Arg150→Cys) and apoE Las Vegas (Ala152→Asp), a finding indicating that the disease may be more common in white populations than initially thought. Results from case studies suggest that fibrates improve renal pathology and may result in the complete clinical remission of the disease. LDL-apheresis or immunoadsorption onto staphylococcal protein A may also have a role in refractory cases.Lipid glomerulopathy is a rare, poorly understood disorder with potentially detrimental consequences. The determination of the effects of apoE mutations on the structural and functional characteristics of the mature protein may provide new insights in the pathogenesis of the disease. Meanwhile, intensive lipid-lowering may reduce proteinuria and preserve renal function in this patient group.