Gastroesophageal reflux disease (GERD) often presents as typical symptoms such as heartburn or acid regurgitation. However, a subgroup of patients presents a collection of symptoms and signs that are not directly related to esophageal damage. These are known collectively as the extraesophageal manifestations of GERD, such as non-cardiac chest pain, laryngitis, chronic cough, hoarseness, asthma or dental erosion. They have a common pathophysiology, involving microaspiration of acid into the larynx and pharynx, and vagally mediated bronchospasm and laryngospasm. The role of extraesophageal reflux in such disorders is underestimated due to often silent symptoms and difficult confirmation of diagnosis. Endoscopy and pH monitoring are insensitive and therefore not useful in many patients as diagnostic modalities. Thus, anti-secretory therapy by proton pump inhibitor is used as both a diagnostic trial and as a therapy in the majority. Attention to optimizing therapy and judicious use of endoscopy and reflux monitoring are needed to maximize treatment success.