Binder syndrome, also known as maxillonasal dysplasia, is an uncommon developmental anomaly. The etiology and pathogenesis of Binder syndrome are still poorly understood, and there is a lack of normative consensus on its treatment strategies. This review retrospectively analyzed relevant studies on the etiology and treatment of Binder syndrome. The main points are summarized as follows: (1) Taken together, the etiology of Binder syndrome can be classified as genetic and non-genetic, whose crucial factor is the inactivation of vitamin K-dependent protein. (2) The management consists of orthodontic treatment, rhinoplasty, and orthognathic surgery. Orthodontic treatment can be performed at any time, while rhinoplasty and orthognathic surgery should be deferred until midfacial growth is almost complete. Therefore, the ideal timing of rhinoplasty is after 14 years old, and for orthognathic surgery, it is after 16-18 years old. Autologous costal cartilage is preferred for rhinoplasty and alloplastic materials are alternatives when donor availability is limited. Maxillary osteotomies should be reserved only for severe patients with Angle Class Ⅲ malocclusion.

Binder syndrome, also known as maxillonasal dysplasia, is an uncommon developmental anomaly. The etiology and pathogenesis of Binder syndrome are still poorly understood, and there is a lack of normative consensus on its treatment strategies. This review retrospectively analyzed relevant studies on the etiology and treatment of Binder syndrome. The main points are summarized as follows: (1) Taken together, the etiology of Binder syndrome can be classified as genetic and non-genetic, whose crucial factor is the inactivation of vitamin K-dependent protein. (2) The management consists of orthodontic treatment, rhinoplasty, and orthognathic surgery. Orthodontic treatment can be performed at any time, while rhinoplasty and orthognathic surgery should be deferred until midfacial growth is almost complete. Therefore, the ideal timing of rhinoplasty is after 14 years old, and for orthognathic surgery, it is after 16-18 years old. Autologous costal cartilage is preferred for rhinoplasty and alloplastic materials are alternatives when donor availability is limited. Maxillary osteotomies should be reserved only for severe patients with Angle Class Ⅲ malocclusion.