Gastroesophageal reflux disease typically manifests as heartburn and regurgitation, but it may also present with atypical or extraesophageal symptoms, including asthma, chronic cough, laryngitis, hoarseness, chronic sore throat, dental erosions, and noncardiac chest pain. Diagnosing atypical manifestations of gastroesophageal reflux disease is often a challenge because heartburn and regurgitation may be absent, making it difficult to prove a cause-and-effect relationship. Upper endoscopy and 24-hour pH monitoring are insensitive and not useful for many patients as initial diagnostic modalities for evaluation of atypical symptoms. In patients with gastroesophageal reflux disease who have atypical or extraesophageal symptoms, aggressive acid suppression using proton pump inhibitors twice daily before meals for three to four months is the standard treatment, although some studies have failed to show a significant benefit in symptomatic improvement. If these symptoms improve or resolve, patients may step down to a minimal dose of antisecretory therapy over the following three to six months. Surgical intervention via Nissen fundoplication is an option for patients who are unresponsive to aggressive antisecretory therapy. However, long-term studies have shown that some patients still require antisecretory therapy and are more likely to develop dysphagia, rectal flatulence, and the inability to belch or vomit.