Objective Current management of fibrous dysplasia (FD) involving the paranasal sinuses and the adjacent skull base is currently controversial. This study aims to present our experience in the management strategy of FD that involves the paranasal sinuses and the adjacent skull base. Methods Twenty three patients from 2006 to 2019 with monostotic fibrous dysplasia (MFD), polyostotic fibrous dysplasia (PFD), or McCune-Albright syndrome (MAS) involving the paranasal sinuses and the adjacent skull base were retrospectively reviewed. This study series was divided into 3 groups based on management strategies: the observation group, the surgery group, and the optic nerve decompression group. Results The observation group included 9 patients with asymptomatic MFD with stable condition during the follow-up period of 15 to 164 months. The surgery group included 10 symptomatic patients with MFD who had personalized endoscopic endonasal surgery. The symptoms of the patients were relieved after surgery. The optic nerve decompression group included 4 patients with visual loss, who underwent endonasal endoscopic optic nerve decompression (EOND) with the aid of image-guided navigation. Their vision improved after surgery. Conclusions Clinical observation and periodic computed tomography (CT) scan are adopted for asymptomatic patients. Surgery is indicated in symptomatic patients. Optic nerve decompression is recommended as soon as possible if the patient has visual loss, whereas prophylactic decompression is not recommended if the optic nerve is encroached by FD without visual loss. Navigation plays an important role in endoscopic surgery involving the paranasal sinuses and the adjacent skull base, especially in FD resection and optic nerve decompression.