Renal diseases have been recognized as a major cause of secondary dyslipidemia since the late 1950's. Two main pathological conditions of renal diseases, impaired renal function and severe proteinuria (nephrotic syndrome), are individually or conjointly associated with altered lipid metabolism depending on the primary diseases. An impaired renal function causes reductions in lipoprotein and hepatic TG lipase activity, the VLDL recep- tor abundance, and ApoC-II to apoC-III ratio, as well as in ApoA-I and LCAT activities. These alterations result in reduced VLDL clearance and the disturbance of HDL synthesis and maturation, leading to uremic dyslipidemia: increased levels of TG, IDL-C, and small-dense LDL-C and decreased levels of HDL-C. Lipid disorders in nephrotic syndrome (NS) are characterized by increased levels of LDL-C and/or TG. NS-induced hypoalbuminemia enhances the synthesis of cholesterol, cholesterol ester, and ApoB, leading to the increased production of LDL and VLDL. Recently, two intriguing molecules were newly identified as inhibitors of lipoprotein clearance. Pro-protein Convertase Subtilisin/Kexin type 9 (PCSK9) is upregulated in NS, and decreases LDL clearance via prompting degradation of the LDL receptor, while angiopoietin-like 4 (Angptl4) is also induced in NS and restricts VLDL clearance via inhibiting lipoprotein lipase. NS impairs HDL maturation from HDL3 to HDL2 due to a reduction of LCAT, with HDL-C levels preserved. Finally, considering that diabetic nephropathy is representative of progressive renal disease and that gluco- corticoids are an anchor drug for the treatment of NS, diabetes- or drug-associated dyslipidemia is occasional- ly superimposed on the original renal dyslipidemia. [Review].