PBX1 and Global developmental delay: Recently, haploinsufficiency of <i>PBX1</i> caused by microdeletions has been shown to result in bilateral renal hypoplasia and other organ malformations.<h4>Materials and methods</h4>Here, we report on a 14-year-old male patient with congenital bilateral dysplastic kidneys, cryptorchidism, hypoplastic clavicles, developmental delay, impaired intelligence, and minor dysmorphic features.