“…The congenital abnormalities of the UPVS is frequently associated with other anomalies, such as biliary tract atresia, polysplenia, and cardiac malformations [Richards and Locksmith, 1998; Massin et al, 1999; Shinkai et al, 2001; Northrup et al, 2002]. A few cases have been reported in association with genetic syndromes: three cases with Noonan syndrome [Leonidas and Fellows, 1976; Currarino et al, 1991; Tordjeman et al, 1996], two cases with the facio‐auricolo‐vertebral spectrum, also known as Goldenhar syndrome [Kalifa et al, 1978; Morse et al, 1986], and one case with Ellis Van Creveld syndrome [Rahemtullah et al, 2001].…”