<p>Glomerulonephritis (GN) is a general term which encompasses various types of glomerular disorders characterized by damage to the capillary endothelium, basement membrane, podocytes, mesangium, or parietal epithelial cells with different combinations leading to proteinuria, hematuria, and azotemia. Although disease process begins in the cells of mentioned above, there is cross-talk with tubular cells leading to tubular atrophy and interstitial fibrosis in the final stages of most GN. Recent developments in genetic, molecular, serologic methods enhances understanding of the pathophysiology and management of GN although more work is needed. The recent ultra-structural studies demonstrated various subcellular disorders present in the context of GN. Mitochondria are one of the most studied subcellular organelles, and various mitochondrial structural and functional alterations have been identified in GNs, including focal segmental glomerulosclerosis, IgA nephropathy, lupus nephritis and anti-glomerular basement membrane disease. However, these studies are still at an early stage and currently the impacts of mitochondrial dysfunction on the development and progression of glomerular disease are not well defined. In the current review article, we examine how mitochondrial dysfunction associates with GN, and discuss the unknowns, conflicting issues and potential treatment options regarding mitochondrial dysfunction and GN.</p>