Background: Allergic fungal rhinosinusitis (AFRS) is a relatively new and incompletely understood clinical entity with characteristic clinical, radiographic, and histopathologic findings. AFRS is often misdiagnosed. Recognition and understanding of this unique disease will lead to efficient diagnosis and treatment of this curable process.; Methods: The following is a review, conducted via a PubMed English language search, of the current diagnosis, pathogenesis, and treatment of AFRS.; Results: AFRS is an immune-modulated disease entity. The Bent and Kuhn diagnostic criteria are the standard for diagnosis of this disease that occurs because of an incompletely understood allergic mechanism. Multimodality treatment relies heavily on surgical therapy along with corticosteroid use and immunotherapy.; Conclusions: AFRS is a unique disease process that differs from other forms of sinusitis and as such requires that physicians understand its diagnosis and management to provide care for patients with this condition.