Osteoid osteoma is a benign bone-forming tumor. Besides, it is small and painful, consisting of osteoid and woven bone, and surrounded by a thick layer of reactive bone formation. The tumor is self-limited or needs to be removed by surgery. This report describes a 16-year-old male with congenital deafness who presented with a right mandibular swelling initiated 2 years ago. The patient underwent ... [Show full abstract] excisional surgery. Histological examinations revealed a benign bone-forming tumor, compatible with osteoid osteoma. Radiologic and histologic correlation confirmed the diagnosis. Base on the diagnosis, the patient received no more treatment. After 4 years of close follow-up examinations, no recurrence was observed in the patient. Osteoid osteoma is often confused with numerous other entities in uncommon sites. Thus, imaging and histologic correlations are required. Proper diagnosis is necessary for further patient management in this respect.