Background: Ossifying fibroma (OF) is benign bone lesions, most frequent in young children, more common in the maxillary sinus and mandible (75-89%), the pathogenesis of the tumor is not clear, there are many subtypes of OF. This paper aims to report an OF a case and literature review., Case Description: Male, 19 years old, with a progressive history proptosis since 2012, diagnosed as a right supraorbital lesion at an external service and assigned to conservative management. Then, he evolved with double vision, which worsened in February of 2018, associated with a moderate headache. On admission: proptosis and downward deviation of the right orbit was noticed on the physical exam and with exception of limited right upgaze, external ocular movements were maintained. Head computed tomography showed a multiloculate expansive osteolytic lesion at the right orbital roof. On magnetic resonance imaging, the lesion had an inner content with septations, T1-weighted imaging heterogeneous signal, T2-weighted imaging high signal intensity, and peripheral contrast enhancement. The patient underwent a right frontal craniotomy with a gross total resection and the postoperative follow-up was uneventful. Menzel reported the first case in 1782. The clinical findings depend on localization. There are five subtypes. In general, the lesions have a radiological appearance with hyperdense boundary and cause deformity and destruction in bones with high recurrence risk. Radical resection is curative., Conclusion: As a result, the correlation of clinical, radiologic, and pathologic data is significant while going for a specific diagnosis in cases of craniofacial fibrous lesions. Total excision is the best treatment, but it can recur., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Surgical Neurology International.)