Introduction: Crouzon syndrome represents an autosomal dominant syndrome, characterized by craniosynostosis. Other features like exopthalmous, proptosis, midface hypoplasia and dental features such as misaligned teeth or dysphasia can be evident. Whilst Peripheral ossifying fibroma is a relatively common reactive gingival growth of uncertain pathogenesis. Case presentation: A 11 years old male child, reported with the chief complaint of discomfort, associated with a growth in the upper right front teeth region. Markedly visible facial and skull asymmetries were also evident along with a short stature. Based on clinical, radiological and histopathological features, and a thorough history, a diagnosis of Peripheral ossifying fibroma in a child with existing Crouzon syndrome was made. Management: Complete surgical excision of the peripheral ossifying fibroma mass was done, and no recurrences were noted. Regarding the Crouzon syndrome, the patient was advised Orthodontic treatment for correcting malocclusion and Dentofacial orthopedics, for managing the skeletofacial symmetries. However the Mother of the child refused any orthodontic intervention. Conclusion: FGFR2 genetic mutation is held responsible for Crouzon syndrome, which needs to be differentiated from other craniosynostoses like Apert syndrome. Peripheral Ossifying fibroma is a nodular, usually ulcerated growth which originates from interdental papilla. Our particular case is unique, because, very few instances of Peripheral ossifying fibromas with existing Crouzon syndrome have been reported in the literature.