This article is focused on the nasal and sinusal manifestations of systemic diseases, such as infections, immunodeficiencies, chronic multisystemic disorders, inflammatory bowel diseases, deposition diseases, hematologic diseases, respiratory diseases, and smell and taste disorders. A concise review of some of the systemic diseases that commonly present complaints in the nose and paranasal sinuses, including their prevalence, sinonasal manifestations, diagnosis, and treatment, is provided.