Objective: The current management of fibrous dysplasia (FD) involving the paranasal sinuses and the adjacent skull base is controversial. This study is to present our experience in the management strategy of FD involving the paranasal sinuses and the adjacent skull base. Design/setting: 23 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 the management strategies: the observation group, the surgery group, and the optic nerve decompression group. Main outcome/results: The observation group included 9 asymptomatic MFD patients with a stable condition during the follow-up period of 15 to 164 months. The surgery group included 10 symptomatic MFD patients having personalized endoscopic endonasal surgery. The patients’ symptoms were relieved after surgery. The optic nerve decompression group included 4 patients with visual loss, who underwent endonasal endoscopic optic nerve decompression with the aid of image-guided navigation. Their vision improved after surgery. Conclusion: The clinical observation and periodic computed tomography (CT) scan is 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 upon 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.