Inflammatory Myofibroblastic Tumor of the Temporal Bone: A Rare Pathology at an Unusual Anatomical Site

Background: Inflammatory myofibroblastic tumor (IMT) is a rare spindle-cell neoplasm that predominantly affects the lungs. Temporal bone involvement is exceptionally uncommon and poses significant diagnostic and therapeutic challenges. Case Report: A 37-year-old female presented with progressive right-sided otalgia, hearing loss, tinnitus and aural fullness for 10 months. Imaging revealed a lesion involving the external auditory canal, middle ear, and mastoid cavity. Histopathology and immunohistochemistry confirmed ALK-positive IMT. The patient underwent sub-total petrosectomy with complete tumor excision. Histopathological examination of the surgical specimen confirmed the diagnosis. Recovery was uneventful, and a follow-up MRI at 3 months showed no residual disease. Conclusion: Temporal bone IMT is a rare entity with non-specific clinical and radiological features. Histopathology with immunohistochemistry is essential for diagnosis, while complete surgical excision remains the treatment of choice. Reporting such rare cases may help improve understanding and guide future management.