Recessive mutations in FREM1 have not been implicated in the development of Fraser syndrome but have been found to cause two rare genetic syndromes, Bifid Nose with or without Anorectal and Renal anomalies syndrome (BNAR; OMIM #608980) and Manitoba OculoTrichoAnal syndrome (MOTA; OMIM #248450), that have significant clinical overlap with Fraser syndrome [19]–[25]. The gene discussed is FREM1; the disease is oculotrichoanal syndrome.