Infantile myofibromatosis is a proliferative disorder characterized by the development of single or multiple nodular lesions in the soft tissue, skeleton, and internal organs. These tumors can occur at any anatomic site, but in one third of the cases, the head and neck region is involved. Here, we report a case of an infantile myofibroma occurring in a 7-year-old girl presented as a solitary lesion in the head and neck area. The clinical heterogeneity and the misleading histopathologic appearances may render the diagnosis difficult. Usually, treatment of choice is surgical removal of the tumor; however, the low rate of recurrence and the possibility of spontaneous regression may lead to conservative surgery or therapeutic abstention.