Renal development process in human is divided into 3 successive stages: pronephros, mesonephros, and metanephros. The tubules continue to mature for 1-2 year after birth. Research of urinary proteome during human renal development is still lacking. Most urine proteome studies focus on postnatal renal maturation period. A comparison between full-term infant and adult urinary protein pattern identified 648 infant-enriched protein spots, of which most were involved in cellular turnover and metabolism. The study of preterm infant urinary proteome compared with term infants suggests elevated IGFBP-1, IGFBP-2, and IGFBP-6, monocyte chemotactic protein-1, CD14, and sialic acid-binding Ig-like lectin 5 during nephrogenesis. Research in several congenital kidney and urinary tract anomalies, ureteropelvic junction obstruction and autosomal dominant polycystic kidney disease, has discovered novel biomarkers, which may help to imply the mechanisms underlying inherited disorders. Future exploration of urinary proteome evolution during renal maturation is needed and will help to find novel biomarkers specially suiting pediatric renal diseases.