BACKGROUND The clinical characteristics of craniomaxillofacial fibrous dysplasia (FD) have not been clearly identified. The objective of this meta-analysis is to assess the predominance of the monostotic form of FD using an evidence-based review. Furthermore, we examined the laterality and sex dominance of FD in patients from international study populations. METHODS We performed a systematic search of PubMed, Embase, Cochrane Central Register of Systematic Reviews, Cochrane Central Register of Controlled Trials and EBSCO for trials published through August 2013. Data extracted from the literature were analysed with Review manager 5.0.24. RESULTS The results of this study showed that unilateral FD occurred more frequently than bilateral FD (RR, 12.37; 95% CI, 2.92-61.24; P = 0.008, N = 263 patients). For unilateral FD, there was no significant difference between cases involving the left or right side of the face (RR, 0.98; 95% CI, 0.66-1.44; P = 0.91; N = 201 patients). There were no significant sex-dependent differences for monostotic and polyostotic forms of craniomaxillofacial FD. CONCLUSIONS There is a significantly higher percentage of the unilateral form than the bilateral form in the craniomaxillofacial FD studies analysed, and an almost equal distribution of left- and right-sided unilateral FD. These proportions were maintained among males and females and there were nearly equal frequencies of monostotic and polyostotic FD.