Myofibroma is a rare benign localized or generalized proliferation of myofibroblastic tissue occurring mostly in infants or children. In the oral region, most lesions occur in the mandible, lip, buccal mucosa, and tongue; however, the lesions arising in the maxilla are very rare. Myofibroma has an aggressive clinical presentation and is often treated aggressively because of an inappropriate diagnosis. A unique feature of central myofibroma of the jaws is the potential for teeth and other odontogenic structures to be involved by tumor. We report a case of myofibroma arising in the left side of the maxilla of a 12-year-old girl and describe the differential diagnosis from other spindle cell lesions of neural and smooth muscle origin. We treated the case using surgical excision under general anesthesia. Immunohistochemical staining was done for establishing the diagnosis since histopathological diagnosis with conventional staining could not distinguish myofibroma from spindle cell tumors.