Inflammatory myofibroblastic tumor (IMT) is a rare tumor composed of mesenchymal myofibroblastic spindle cells enveloped by an inflammatory infiltrate. Historically, this tumor sparked debate regarding whether it was a true malignancy with metastatic potential or merely a locally destructive physiologic inflammatory response. Few reports of IMT exist in the recent literature, with the majority of cases occurring in the pediatric population and favoring the lungs. Here we present an exceedingly rare case of IMT involving the larynx of a 22-year-old female. A hemorrhagic and solid mass of the right true membranous vocal fold was excised and sent for histopathological assessment. Features of the surgical specimens were diagnostic for IMT. Intralesional steroid therapy was selected for additional treatment. Panendoscopy facilitated surveillance for any additional or recurrent lesions, of which there were none. At 11 months post-excision, follow-up MRI revealed symmetric vocal cords without evidence of any masses. Although rare, laryngeal IMT should be considered in any patient presenting with hoarseness due to a vocal fold mass. Based on the successful treatment of our patient, we suggest that our approach of surgical excision followed by intralesional corticosteroid injection may be an efficacious treatment approach for this rare tumor. However, more research is warranted to elucidate the most effective, safe, and cost-effective treatment approach. Copyright © 2021 The Voice Foundation. Published by Elsevier Inc. All rights reserved.