Infantile myofibromatosis is a rare proliferative mesenchymal disorder that has a potential for rapid growth and recurrence. Approximately 30% of infantile myofibromatosis lesions present in the head and neck, and some of these can cause significant morbidity. We report a case of recurrent infantile myofibromatosis that persisted after surgery and chemotherapy and was managed conservatively. Treatment of infantile myofibromatosis varies according to the size, location, and extent of disease. Solitary lesions may be simply observed in view of their potential for regression, but surgical excision, radiotherapy, and/or chemotherapy should be considered on a case-by-case basis, especially for nonresectable, rapidly progressive, or symptomatic lesions.