In summary, in view of the straightforward genetic diagnosis, we recommend EIF2AK3 mutation screening for diagnosis in all neonatal/early-onset diabetes patients (at least before age of 6 months) with clinical features specific for WRS, or in the absence of extra-pancreatic manifestations in PNDM patients born from consanguineous parents. Here, EIF2AK3 is linked to Wolcott-Rallison syndrome.