The sinonasal cavities represent an anatomical region affected by a variety of tumours with clinical, aetiological, pathological, and genetic features distinct from tumours at the main head and neck cancer localizations. Together, squamous-cell carcinoma and adenocarcinoma account for 80% of all sinonasal tumours, and are aetiologically associated with professional exposure to wood and leather dust particles and other industrial compounds, and therefore, are officially recognized as an occupational disease. Owing to their distinctive characteristics, sinonasal tumours should be considered as separate entities, not to be included in the miscellany of head and neck cancers. Sinonasal tumours are rare, with an annual incidence of approximately 1 case per 100,000 inhabitants worldwide, a fact that has hampered molecular-genetic studies of the tumorigenic pathways and the testing of alternative treatment strategies. Nevertheless, the clinical management of sinonasal cancer has improved owing to advances in imaging techniques, endoscopic surgical approaches, and radiotherapy. Genetic profiling and the development of in vitro cell lines and animal models currently form the basis for future targeted anticancer therapies. We review these advances in our understanding and treatment of sinonasal tumours.