Bowel obstruction is a common cause for admission into the NICU, but pyloric atresia (PA) is a very rare cause of bowel obstruction. This article illustrates the development of the fetal gastrointestinal tract, most specifically the stomach and pylorus. Pathophysiology, typing, and treatment of PA are also explored. Presented are two cases of PA that occurred in a Level III NICU one month apart. Management of this condition is surgical in nature. Long-term prognosis is usually excellent because this defect is often isolated.