,42 At present, we support the previously proposed term ‘type IV collagen (COL4A3/COL4A4) nephropathy’ as a broad and overlying diagnosis for all patients with at least glomerular hematuria and a mono-allelic variant COL4A3 or COL4A4. 43In the future, an accurate molecular diagnosis of kidney disorders related to COL4A3/COL4A4 variants and accompanying modifying genes might further contribute to the classification of these kidney diseases and will have implications for monitoring, treatment, and genetic counseling of patients and at-risk family members. Here, COL4A3 is linked to Nephropathy.