Type IV collagen-related nephropathies are a genetically diverse group of inherited kidney disorders caused by mutations in the collagen type IV alpha-3 (COL4A3), alpha-4 (COL4A4) or alpha-5 (COL4A5) genes, which encode essential components of the glomerular basement membrane (GBM) [1]. The gene discussed is COL4A5; the disease is kidney disorder.