In 2004, Mitchell and colleagues initially reported Mitchell-Riley syndrome (MTCHRS) in five infants from Pakistani and Asian parents presented with hypoplastic or annular pancreas, jejunal and duodenal atresia, neonatal diabetes, and gallbladder aplasia or hypoplasia (Mitchell et al. 2004). The triad of presentations include neonatal diabetes, gallbladder agenesis/hypoplasia, and intestinal atresia (Alruqaie and Alfadhel 2019). The frequency of neonatal diabetes is estimated to be 1 per 90,000–160,000 births, but the occurrence of MTCHRS is less (Concepcion et al. 2014). The age of onset is usually during neonatal period (Kambal et al. 2019).