Rarer individuals with classical features of UFS have biallelic variants in LRIG2 (UFS2; Mendelian Inheritance in Man #615112), encoding a plasma membrane associated protein called leucine rich repeats and immunoglobulin like domains 2 (Stuart et al., 2013; Fadda et al., 2016; Sinha et al., 2018). The gene discussed is LRIG2; the disease is Ochoa syndrome.