Lipoprotein glomerulopathy (LPG) is a rare disease characterized by specific histological, immunomorphological, and ultrastructural changes. The main pathomorphological signs of LPG are lipoprotein thrombi in the lumen of the capillary loops, proteinuria, and dyslipoproteinemia as an increased concentration of apolipoprotein E (phenotypes E2/E3, E2/E4). A patient aged 47 years had nephrotic syndrome with a daily protein loss of 12.4 g/ day. Light optical microscopic examination of a renal biopsy specimen revealed Sudan III-positive, weakly PAS, and eosin-positive friable thrombi in the lumen of the capillary loops. No deposits of immunoglobulins were immunomorphologically detected in thrombotic masses. The ultrastructure of thrombotic masses was composed of granular masses with numerous vacuoles that formed concentric layers. Thus, LPG was diagnosed as a manifestation of type 3 hyperlipoproteinemia that had characteristic pathological, clinical, and laboratory signs of this disease.