Objective To explore the surgical treatment strategy for optic canal stenosis caused by craniofacial fibrous dysplasia.Methods A retrospective analysis was made reviewing the 18 cases from August 2012 to September 2017.The clinical and imaging findings and the discussion of the operative methods and results were summarized.Results Seven patients were treated with optic nerve decompression,five patients received prophylactic decompression,and six patients received fibrous dysplasia resection.At the end of follow-up,among patients who received optic nerve decompression,3 patients achieved visual improvement,7 maintained previous vision and 2 had decreased visual acuity.Conclusion Optic nerve decompression can effectively terminate the trend of visual deterioration and possibly improve visual acuity for patients suffered from optic canal stenosis caused by craniofacial fibrous dysplasia.Neuronavigation can effectively improve the efficiency and safety of operation,and reduce the risk of optic nerve injury and visual deterioration.