Helicobacter pylori is a worldwide common bacteria that infects humans. This Gram-negative microorganism is microaerophilic, spiral or curved shaped, and urease, catalase and oxidase-positive. It has the ability to live in the acidic environment of the gastric mucosa. It has been shown that H. pylori plays a role in the development of gastric ulcers and malignant lesions. Furthermore, it was reported that H. pylori may be a cause of several systemic illnesses such as cardiovascular, dermatologic, immunologic, neurologic, hematologic, ophthalmologic, gynecologic, endocrine, and hepatobiliary diseases. In addition, positive or negative correlations between H. pylori infection and rhinitis, sinusitis, adenoiditis or adenoid hyperplasia, otitis media, tonsillitis, or tonsil hypertrophy have been demonstrated in various studies in the literature. However, H. pylori's role in the pathogenesis or association with these diseases remains controversial. Some studies reported that systemic immune and inflammatory responses against H. pylori might cause some systemic diseases as well as different types of malignancies. Although there are studies about the role of H. pylori in benign and malignant diseases of the upper and lower respiratory tract, further studies are needed to reveal the pathophysiological relationship between H. pylori infection and respiratory diseases. The aim of this review was to summarize the studies that reported either a positive or negative relationship between H. pylori and benign and malignant diseases of the respiratory tract.