G6PD and cholestasis: Here we describe a male newborn who met the HLH diagnostic criteria, presented with profound cholestasis, and carried a maternally inherited heterozygous mutation in <i>syntaxin-binding protein-2</i> [<i>STXBP2</i>, c.568C>T (p.Arg190Cys)] in addition to a severe pathogenic variant in <i>glucose 6-phosphate dehydrogenase</i> [<i>G6PD</i>, hemizygous c.1153T>C (Cys385Arg)].