Inflammatory myofibroblastic tumor is an uncommon lesion which mainly develops in the lung and is extremely rare in the larynx. It may be easily misinterpreted as a malignant epithelial or mesenchymal spindle cell neoplasm. Histological and clinical knowledge of this lesion is important to exclude misdiagnosis and inappropriate treatment. We report a case of inflammatory myofibroblastic tumor arising on the right vocal cord of a 23-year-old man. The tumor was composed of a mixture of spindle cells and inflammatory elements. Immunohistochemical investigation revealed that the neoplastic cells expressed anaplastic lymphoma kinase (ALK) protein.