Type II citrullinemia has two main clinical phenotypes: neonatal intrahepatic cholestatic hepatitis caused by citrin deficiency (NICCD; OMIM# 6,05,814) and adult-onset type II citrullinemia (CTLN2; OMIM# 6,03,471) (Saheki and Kobayashi, 2002). Here, SLC25A13 is linked to neonatal intrahepatic cholestasis due to citrin deficiency.