<p>Juvenile ossifying fibroma (JOF) is a rare fibro-osseous tumor affecting the craniofacial bones, characterized by its benign yet potentially aggressive nature, with a distinct onset at an early age. Fibrous dysplasia, a fibro-osseous lesion, involves the replacement of normal bone with disorganized fibrous tissue and accounts for 2% of osseous tumors. We report a case of a 14-year-old girl diagnosed with both juvenile ossifying fibroma of the maxilla and juvenile Paget’s disease (JPD), a combination that has not been previously documented. The coexistence of these two conditions highlights the complex relationship between these distinct pathologies. This 14-year-old girl presented with a one-month history of swelling in the right mid-facial area, accompanied by pain. Radiographic findings revealed an expansile lesion in the maxilla consistent with JOF, as well as hyperostosis throughout the skull, which aligns with the radiographic features of Paget’s disease. Histopathological examination confirmed the diagnosis of trabecular ossifying fibroma. The tumor was completely excised, followed by reconstruction of the orbital floor. This case emphasizes the importance of considering the possibility of concurrent disorders in pediatric patients and underscores the need for comprehensive diagnostic evaluation and a multidisciplinary approach in managing complex bone conditions. The interaction between JOF and JPD may influence treatment strategies and outcomes, necessitating a personalized approach to patient care.</p>