Case Report A 3-year-old girl presented to the maxillofacial clinic with an asymptomatic large lump on the left side of the mandible with failure of teeth to erupt in the area. She presented with a firm, nontender swelling of the lower left canine region exhibiting no signs of infection. Furthermore, her lower left deciduous canine was missing despite normal dental development elsewhere in the mouth. Computed tomography scan of the mandible revealed an expansile, cystic lesion closely associated with an unerupted mandibular deciduous canine. Odontogenic tumors with analogous clinical and radiographical presentations as described include keratocysts, ameloblastomas and odontogenic myxomas. An incisional biopsy was performed to determine a definitive diagnosis where histology revealed a central odontogenic fibroma (COF). In particular, the nest of odontogenic epithelium in between the fibroblastic stroma was pathognomonic for COF. Subsequently, she underwent complete enucleation of the mass, curettage of the residual bone, and extraction of the enveloped deciduous tooth. Clinical Relevance COFs are an extremely rare group of benign neoplasms accounting for <1.5% of all odontogenic tumors. Such distinctions between the differential diagnoses are vital because several of the aforementioned tumors have high recurrence rates, whereas COF classically does not. Furthermore, the age of the patient here is remarkable because the literature describes the mean age of presentation as 31 years old. Conclusions This atypical case of COF highlights the importance of accurate histologic findings, which are pertinent to its diagnosis. It also incorporates several specialties and emphasizes the importance of early detection, diagnosis, and treatment. A 3-year-old girl presented to the maxillofacial clinic with an asymptomatic large lump on the left side of the mandible with failure of teeth to erupt in the area. She presented with a firm, nontender swelling of the lower left canine region exhibiting no signs of infection. Furthermore, her lower left deciduous canine was missing despite normal dental development elsewhere in the mouth. Computed tomography scan of the mandible revealed an expansile, cystic lesion closely associated with an unerupted mandibular deciduous canine. Odontogenic tumors with analogous clinical and radiographical presentations as described include keratocysts, ameloblastomas and odontogenic myxomas. An incisional biopsy was performed to determine a definitive diagnosis where histology revealed a central odontogenic fibroma (COF). In particular, the nest of odontogenic epithelium in between the fibroblastic stroma was pathognomonic for COF. Subsequently, she underwent complete enucleation of the mass, curettage of the residual bone, and extraction of the enveloped deciduous tooth. COFs are an extremely rare group of benign neoplasms accounting for <1.5% of all odontogenic tumors. Such distinctions between the differential diagnoses are vital because several of the aforementioned tumors have high recurrence rates, whereas COF classically does not. Furthermore, the age of the patient here is remarkable because the literature describes the mean age of presentation as 31 years old. This atypical case of COF highlights the importance of accurate histologic findings, which are pertinent to its diagnosis. It also incorporates several specialties and emphasizes the importance of early detection, diagnosis, and treatment.