The term "giant hiatus hernia" has been variably defined, but most authors refer to at least 30% of the stomach, with or without other abdominal viscera, herniating through the oesophageal hiatus of the diaphragm into the mediastinum. This causes dysfunction of the lower oesophageal sphincter complex and can lead to obstructive symptoms, atypical extra-oesophageal symptoms as well as medically difficult to control reflux. When indicated, operative repair involves complete reduction of the hernia and the associated peritoneal sac, and partial hiatal closure in combination with an anti-reflux procedure. Surgery for giant hiatal hernias is complex, but can be performed with low morbidity and mortality with effective, long-term symptom resolution and improvement in health related quality of life.