In an individual with biallelic variants in LRIG2, the causal gene for urofacial syndrome (MIM 615112), and a homozygous LIG4 missense variant, the affected child had recurrent episodes of urosepsis, secondary to severe vesicoureteral reflux, leading to left kidney hypoplasia and scarring (Fadda et al., 2016). The gene discussed is LIG4; the disease is vesicoureteral reflux.