The present work aims to report a case of dentinogenic ghost cell tumor (DGCT) associated with compound odontoma (CO). An 8-year-old patient was referred to the oral medicine discipline presenting with dental retention. Computerized tomography (CT) scan revealed a well-defined unilocular radiolucent lesion involving an irregular mass, associated with an unerupted permanent upper central incisor (tooth 21). Excisional biopsy was performed. Histopathologic analysis revealed enamel matrix, loose connective tissue resembling dental pulp, enveloped in a fibrous capsule, associated with odontogenic epithelium sheets presenting aberrant keratinization, and containing abundant dentinoid and osteoid material. Based on clinical and histopathologic findings, the final diagnosis was DGCT and CO. Since spontaneous eruption failed to occur within the expected time frame (6 months’ follow-up), surgical exposition of the crown and bonding of an orthodontic appliance for traction was done to facilitate eruption. Up to now, the patient is under follow-up care in the orthodontics clinic.