Among these features, genital anomalies and developmental delay were significantly more frequent in CAKUT patients with versus without rare TSHZ3 variants (5/12 (42%) versus 44/291 (15%), p = 0.029, and 3/12 (25%) versus 17/291 (6%), p = 0.037, respectively, two-tailed Fisher’s exact test). This evidence concerns the gene TSHZ3 and Global developmental delay.