The normal architecture of the kidney is crucial for maintaining biological function and its homeostasis. Its proper development depends on highly dynamic processes which modulate the integrity of its associated cellular functions, interactive events and regulatory cascades altogether providing proper turnover in adult life. Any alteration in regulatory processes and normal utility holds crucial consequences for proper functioning of the kidney. These variations accompany renal injury, various etiologic events, deviation from genetic-wild type processes and metabolic disturbances leading to major lesion of end-stage renal disease (ESRD). Major renal disorders developing today affecting millions globally include diabetes, hypertension, glomerulonephritis and Polycystic Kidney Disease (PKD). Among these diseases, PKD is becoming relatively common, and has emerged as one of the largest causes of renal transplantation and dialysis. Therefore, understanding the development, function and progression of normal kidneys to cystic renal kidneys serves an important way in understanding pathophysiology of PKD and cystogenesis.