Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 6 1 2021 05 15 6 10 Hedieh Moradi Tabriz Department of Pathology, Sina Hospital, Tehran University of Medical Sciences, Tehran, Iran. Elham Nazar Department of Pathology, Sina Hospital, Tehran University of Medical Sciences, Tehran, Iran. Arezo Efekhar Javadi Department of Pathology, Sina Hospital, Tehran University of Medical Sciences, Tehran, Iran. 2020 06 14 2020 07 31 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 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. https://crcp.tums.ac.ir/index.php/crcp/article/view/364 https://crcp.tums.ac.ir/index.php/crcp/article/download/364/389