The observed prevalence of NICCD in Japan is similar to calculated homozygous and compound heterozygous carrier rate (1:17000) [1, 8], and over 80,000 East Asians are estimated to be homozygous for SLC25A13 gene pathogenic variants [6]. This evidence concerns the gene SLC25A13 and neonatal intrahepatic cholestasis due to citrin deficiency.