A 1-day-old girl, late preterm (36 weeks 3 days) infant, was admitted to the neonatal intensive care unit for respiratory distress and megacystis seen on a prenatal ultrasound at 24 weeks of gestation. A babygram (anteroposterior view of the entire body) showed absence of bowel gas, and abdominal ultrasound revealed grade 4 bilateral hydronephrosis. The patient was subsequently diagnosed with megacystis-microcolon–intestinal hypoperistalsis syndrome and was taken to the operating room for appropriate treatment.