Chronic sphenoid rhinosinusitis is a spectrum of inflammatory diseases in isolated sphenoid sinus which may persist over a period of 12 weeks. It is a different entity from other types of rhinosinusitis because clinical presentations include headache, visual loss or diplopia, and patients may or may not have nasal obstruction or nasal discharge. Nasal endoscopic examination is useful, and computed tomography is mandatory. The disease requires comprehensive knowledge and appropriate imaging technique for diagnosis. To treat patients with chronic sphenoid rhinosinusitis, surgical treatment with endoscopic transnasal sphenoidotomy is often required. As there are no recent updated reviews of chronic sphenoid rhinosinusitis, in this article, we review the anatomy of the sphenoid sinus and its clinical relationship with the clinical signs and symptoms of the disease, the imaging findings of each diagnosis and the comprehensive surgical techniques.