A giant hiatal hernia (HH) is a hernia that includes at least 30% of the stomach in the chest, although a uniform definition does not exist; most commonly, a giant HH is a type III hernia with a sliding and paraesophageal component. The etiology of giant HH is not entirely clear, and two potential mechanisms exist: (1) gastroesophageal reflux disease (GERD) leads to esophageal scarring and shortening with resulting traction on the gastroesophageal junction and gastric herniation; and (2) chronic positive pressure on the diaphragmatic hiatus combined with a propensity to herniation leads to gastric displacement into the chest, resulting in GERD. The short esophagus and GERD are key concepts to understanding the pathophysiology of giant HH, and these concepts are critical to address this problem appropriately. A successful repair of giant HH requires adherence to basic hernia repair principles (ie, hernia sac excision, tension-free repair), recognition and correction of a short esophagus, and a well-performed antireflux procedure. Recurrence rates for open giant HH repairs in expert hands range between 2% and 12%; large series have demonstrated that meticulous laparoscopic surgical technique can emulate the results of open giant HH repair.