Among of these patients, the smallest overlapping region (SRO) focus on PBX1 gene, which is proposed to be relevant to syndromic CAKUT [8]; In addition, Laurence et al. identified five de novo heterozygous loss of function mutations in PBX1 gene or microdeletions involving the PBX1 gene in 204 unrelated CAKUT patients [11]. Here, PBX1 is linked to congenital anomaly of kidney and urinary tract.