Myxoma of the jaw is a rare, benign bone tumor of odontogenic origin. Given the locally aggressive nature of the myxoma and the high rate of recurrence, there is a tendency to choose radical resection of the jaw segment containing the tumor mass as the treatment modality. As they appear to attain a considerable size prior to diagnosis because of their insidious growth characteristics, particularly in the maxilla, treatment requiring a hemimaxillectomy often results in a significant functional and aesthetic disability. This is particularly distressing as this condition usually affects the young. We present the case of a 16-year-old female who suffered a large maxillary myxoma with swelling of the left cheek for 2 years. Wide resection of the tumor tissue including the maxillary medial and lateral buttresses and part of the hard palate with preservation of maxillary alveolar bone and teeth was undertaken, and the maxillary buttresses were reconstructed with autogenous rib grafts. At the 3.5-year follow-up there was no local recurrence and no tooth loss. Both the functional result and aesthetic contour proved satisfactory. Wide resection with preservation of vital structures and simultaneous autogenous bone graft reconstruction is our preferred method. A long-term follow-up is needed.