Background: Craniosynostosis is a congenital deformity of the skull that occurs at birth. Objectives: This study aimed to investigate the frequency of different types of craniosynostosis in Isfahan, Iran. Methods: In this retrospective study, the records of all patients referred to the pediatric neurosurgery clinic of Imam Hossein children’s hospital in Isfahan, Iran from 2013 to 2018 were diagnosed with craniosynostosis. Incomplete files were excluded. Demographic information of patients and type of delivery, underlying diseases of their parents, diseases of maternal pregnancy, type of craniosynostosis, syndromic or non-syndromic craniosynostosis were collected. Data were analyzed using SPSS software version 22. Results: The diagnosis of craniosynostosis in 63% of cases (n=121) was a single suture and in 37% of cases (n=72) was a complex suture. There was no significant difference in the frequency distribution of craniosynostosis in males and females (P>0.05). Conclusion: Due to the prevalence of 16 cases per 100,000, we can reduce the complications of this disease by educating doctors and parents.