Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal disease of low malignancy consisting of inflammatory cells inside a mesenchymal stroma comprising myofibroblasts. Biological behavior is variable, recurrence is uncommon, metastatic disease is rare. Treatment strategy is based on the status of low-grade malignant tumor. Radical surgery is considered to be the principal treatment modality, except if it requires a mutilating procedure. Only 6 cases of pediatric patients treated for IMT of the esophagus have been reported in English-language literature. Herein, a case of a 13-year-old boy with an IMT of the esophagus is presented.