Myofibromatosis generally is regarded as a fibrous proliferative disease of neonates and children that, even when multicentric, has an excellent prognosis and a propensity for spontaneous involution. Reported is a case of solitary, locally recurrent myofibromatosis in the ear canal of an adult patient. It was initially thought to be a leiomyoma. The diagnosis of myofibromatosis was made on the basis of its histologic appearance and corroborated by immunohistochemical stains. Clinicians and pathologists should be aware that myofibromatosis may occur in adults in unusual locations, with a potential for local recurrence. Furthermore, its histologic appearance may mimic that of other mesenchymal neoplasms.