A 3-month-old Arab female infant presented with neonatal DM, epiphyseal dysplasia, and liver failure; she was diagnosed with Wolcott-Rallison syndrome caused by the pathogenic homozygous mutation of c.1293G > A (p.W431*) in EIF2AK3, which was previously reported to be pathogenic [29]. Here, EIF2AK3 is linked to Wolcott-Rallison syndrome.