Manifestations of atypical gastroesophageal reflux disease (GORD) are varied, and the presentation of atypical symptoms may occur in the absence of typical symptoms. The most sensitive and specific investigation for GORD is pH monitoring, and its application in atypical disease is utilized throughout this paper as a basis for correlating disease and pathogenesis. The less well-known areas of laryngeal manifestations, particularly chronic hoarseness and globus, are discussed in addition to recent work on orodental manifestations. Well-known areas of cardiac and respiratory manifestations, which include chronic cough and asthma, are also reviewed. Evidence from clinical trials indicates that aggressive anti-reflux therapy in patients with atypical symptoms can be effective. Where appropriate, medical therapy may involve long-term proton pump inhibitor, although further research outlining the roles of other therapies such as surgery is awaited.