Background: Antley-Bixler syndrome (ABS) is an extremely rare genetic disorder characterized by distinct features such as trapezoidal face craniosynostosis, midface hypoplasia with exorbitism, depressed nasal bridge, chonal atresia, radio-humeral synostosis, joint contractures and arachnodactyly.

Objective: The aim of our study is to: i) Comprehensively analyse available literature on ABS and report on the surgical management as well as treatment outcomes. ii) Describe a case of ABS that has been successfully treated with monobloc distraction osteogenesis via virtual surgical planning.

Methods: A PUBMED search was performed in June 2024. The search was based on a general search string limited to "Antley-Bixler". Inclusion criteria included systematic reviews preferably with meta-analysis, randomized controlled trials, cohort studies and case reports that were reported in English.

Results: Papers that focussed on surgical techniques were only present in the form of case reports and case series, out of which only 8 papers that fulfilled the inclusion criteria were included. Surgical techniques that were reported on included fronto-orbital advancement, midfacial advancement, distraction osteogenesis and others. The reported patients also underwent several other surgical procedures for either functional or aesthetic purposes. All the patients reported a good outcome from their surgical interventions.

Conclusions: Monobloc distraction osteogenesis is one of the most reliable and predictable procedures that may solve various problems associated with complex craniofacial deformities such as in ABS. This can be seen from the favourable outcome reported in several studies. However, comprehensive pre-surgical virtual planning and multidisciplinary care contributes greatly to favourable functional outcomes.