Thymic lesions comprise approximately 2-3% of all pediatric mediastinal tumors and include thymic cysts, hyperplasia, carcinoma, and thymomas. Thymomas, which represent less than 1% of all mediastinal tumors, are rare mediastinal tumors in the pediatric population. Fewer than 30 cases in children have been described in the literature. These tumors are typically aggressive, with poor outcomes. We report a thymoma in a 14-year-old girl and review the available literature on thymomas and their treatment.