Purpose/Aim: Paraesophageal hiatus hernias are seldom found, however the incidence is increasing accounting for 5-10% of all hiatal hernias. The aim of this review is to emphasize controversies in clinical presentation, essential workup investigations and highlight non-surgical and surgical management options. Materials and Methods: A PubMed literature search using the keywords 'large or giant paraesophageal hernia', 'hiatus or hiatal hernia', 'laparoscopic surgery', 'antireflux surgery', 'mesh', 'gastric volvulus' and 'diaphragmatic hernia' published between 1998 until 2017 was conducted. Results: Presenting symptoms are non-specific and can be erroneously attributed to various more common medical conditions. Significant complications as gastric volvulus and stomach necrosis, may occur and the obscured clinical presentation can be confusing for the clinician. Management options in the elective setting are controversial, and surgical repair cannot be easily justified for a minimally symptomatic condition, especially in an elderly and perhaps frail patient. However, in the era of laparoscopic surgery around the hiatus, reduced operative stress makes surgical repair appealing in the elective setting. Surgical matters as the adjunct of an antireflux procedure or not, the use of prosthetic mesh to reinforce the hiatus, gastropexy and the clinical importance of radiological or endoscopic recurrence are still under debate. Conclusions: The laparoscopic treatment of paraesophageal hiatus hernias is effective with low morbidity rates, offered in symptomatic patients and good operative risk asymptomatic individuals. More studies are needed to assess improvement suggestions, as the use of prosthetic mesh or gastropexy, regarding complications and recurrence risks.