Parry-Romberg syndrome (PRS) is a rare craniofacial disorder whose etiology has not been well understood. The objective of this study was to evaluate the sex difference and the scope of female predominance in PRS. The study used meta-analysis to examine 838 diverse patients from 26 articles. The result of the study showed a significantly higher risk in women in the PRS study populations; the pooled female ratio was 2.23 (95% confidence interval, 1.77–2.80; P < 0.00001; N = 838 patients). There was no significant difference in the association between laterality of PRS and sex, wherein female and male patients have almost the same distribution of left- and right-sided PRS.