SIGNIFICANCE: Autophagy is emerging as an important pathway in many biological processes and diseases. This review summarizes the current progress on the role of autophagy in renal physiology and pathology. RECENT ADVANCES: Studies from renal cells in culture, human kidney tissues, and experimental animal models implicate that autophagy regulates many critical aspects of normal and disease conditions in the kidney, such as diabetic nephropathy and other glomerular diseases, tubular injuries, kidney development and aging, cancer, and genetic diseases associated with the kidney. CRITICAL ISSUES: The importance of autophagy in the kidney has just started to be elucidated. How the process of autophagy is altered in the pathogenesis of kidney diseases and how this alteration is beneficial or detrimental to kidney functions still need to be fully understood. FUTURE DIRECTIONS: Investigations that uncover the precise mechanism and regulation of autophagy in various kidney diseases may lead to new strategies for therapeutic modulation.