In comparison, our data considering the specific variant COL4A3 c.2881+1G>A showed that at the time of biopsy or first clinical evaluation, hematuria was present in 97.1%, proteinuria in 55.9%, hearing loss in 17.6%, and ocular abnormalities in 11.8%, whereas at the time of the last follow-up, 30 (88.2%) patients had hematuria, 23 (67.6%) had proteinuria, and 5 (14.7%) developed KF, started dialysis, or underwent kidney transplantation by the median age of 48 years (age range 27–55 years). The gene discussed is COL4A3; the disease is Hematuria.