Intrathoracic gastric volvulus associated with neonatal paraesophageal hernia is very rare in the newborn period. We report a case of a 3-week-old term infant who presented to the hospital with a history of non-bilious vomiting. Workup for hypertrophic pyloric stenosis eventually revealed the presence of a congenital hiatal hernia and intrathoracic gastric volvulus requiring urgent surgical management. The infant underwent successful laparoscopic repair. We discuss the diagnosis and management of this extremely rare surgical cause of neonatal nonbilious emesis.