Mandibular distraction osteogenesis has become an important surgical option in children with developmental micrognathia, Pierre Robin sequence (PRS) and other syndromic micrognathias. Upper airway obstruction in children born with PRS and syndromes such as Nager syndrome and Treacher Collins can often be managed conservatively with prone positioning, nasopharyngeal airways, or continuous positive airway pressure devices. However, for children who have failed these conservative methods, surgical treatment needs to be considered. Surgical options include a variety of procedures, but the most described include tongue lip adhesion, mandibular distraction osteogenesis and ultimately tracheostomy. We conducted a systematic review of mandibular distraction in children with upper airway obstruction secondary to micrognathia. The search strategy was designed to find both published and unpublished studies since 1990, and the databases searched included PubMed, Embase, Scopus, Web of knowledge and grey literature sources. The inclusion criteria includes studies in children with clinical evidence of micrognathia/PRS who have failed conservative treatments, including both syndromic and non-syndromic PRS. The data extracted from the included studies includes the effect of mandibular distraction osteogenesis on the following outcomes including airway patency, decannulation of tracheostomized patients, gastro-oesophageal reflux, feeding and long term outcomes. The results of surgical outcomes including surgical protocol, rate of early re-operation and complications were also collected where available in the studies included in this analysis. The results of this systematic review will be presented with the objective of answering the question: What is the effectiveness of mandibular distraction on airway patency in children born with Pierre Robin Sequence?