Progressive hemifacial atrophy (PHA) is a self-limited atrophy of subcutaneous tissues (and less frequently of hard tissues) on one side of the face. It is a sporadic, slowly progressing disease whose pathogenesis is still unknown. As a rule the asymmetry caused by PHA (usually of soft tissue) is treated by volume augmentation that involves free tissue grafting or a pedicled flap transfer.We describe a rare case of hard tissue PHA. The atrophic changes occurred in the left molar tooth, maxilla and mandible and were associated with moderate soft tissue atrophy. The left side of the patient's mouth was higher on the right side, and the occlusal plane was severely inclined in spite of normal occlusion. After no alteration and no progression of the atrophy were established, the patient was treated with orthodontic surgery. To correct the inclined occlusal plane and asymmetry profile, we performed a Le Fort I and intraoral vertical ramus osteotomy. In the 3-year follow-up, there were good occlusal balance and improved symmetrical profile without relapse or recurrence of the atrophy. Thus, orthodontic surgery was effective as a first procedure to treat hard tissue atrophy that appeared with moderate soft tissue atrophy.