This study aimed to investigate the genetic features, clinical manifestations, and genotype-phenotype correlations in Chinese children with <i>COL4A4</i> variants.<b>Methods:</b> 19 children with <i>COL4A4</i> variants who presented with hematuria and/or proteinuria as their main complaints were included in our analysis. The gene discussed is COL4A4; the disease is Hematuria.