Citrin deficiency exhibits several age-dependent clinical manifestations: in newborn and infant stages, it could cause neonatal cholestasis, defined as conjugated hyperbilirubinemia in the newborn period or shortly thereafter, therefore referred to as Neonatal Intrahepatic Cholestasis (NICCD: OMIM 605,814); in older children, deficiency of citrin could exhibit as Failure to Thrive and Dyslipidemia (FTTDCD); in adults, it causes type II Citrullinemia (CTLN2: OMIM 603,471).2 Here, SLC25A13 is linked to citrullinemia type II.