Maxillary skeletal prognathism can involve severe mandibular micrognathia with marked mandibular retrognathism or hypoplasia. For patients with such a condition, a conventional treatment is mandibular advancement by sagittal split ramus osteotomy (SSRO). This procedure has problems such as insufficient advancement, instability of jaw position, and postoperative relapse. Thus, in recent years, mandibular distraction osteogenesis has been used in some patients. Mandibular distraction has many advantages, but an ideal occlusion is difficult to achieve using this procedure. That is, 3-dimensional control cannot be attained using an internal device that is unidirectional. This report describes a case of severe mandibular micrognathia in a 14-year-old girl treated using backward distraction osteogenesis. This procedure was first reported by Ishii et al (Jpn J Jaw Deform 2004; 14:49) and involves a combination of SSRO and ramus distraction osteogenesis. In the present study, intermaxillary fixation in centric occlusion was performed after osteotomy, and proximal bone segments were distracted in a posterosuperior direction. This procedure is a superior surgical technique that avoids the drawbacks of SSRO and conventional mandibular distraction. However, it applies a large load to the temporomandibular joints and requires thorough management. Thus, careful evaluation needs to be made of the indication for backward distraction osteogenesis.