Alport syndrome (AS) is a rare hereditary progressive kidney disease which often leads to end stage renal disease and irreversible damage of kidney function. It is clinically characterized by progressive nephritis, hematuria, sensorineural hearing loss and abnormalities of the eye. This disease is caused by mutation of the COL4A3, COL4A4 or COL4A5 genes, which results in the synthesis of the irregular type IV collagen, structurally significant protein of the basement membranes in kidney, ear and eye. The disease is most often (85%) inherited by the X-linked mutation of the COL4A5 gene. In this AS type (XLAS) men are presented with more severe disease compared to women, with extrarenal symptoms and early onset of end stage renal disease, usually before age of 30. Only 15% of all AS types develop as a result of COL4A3/COL4A4 autosomal mutation, which can be inherited recessively, or far less often, dominantly. ARAS is clinically presented similar to male XLAS patients, while clinical presentation of ADAS can vary, regardless of the affected sex, from isolated hematuria to end stage renal disease (ESRD). The disease is progressive and incurable, but it is well known that ACE inhibitors can slow down the progression of the disease and delay terminal stage of kidney failure.