The Pierre Robin sequence is defined as a congenital anomaly characterized by the presence of glossoptosis, micrognathia, and airway obstruction, leading to various complications. Mandibular distraction osteogenesis (MDO) emerges as a surgical treatment option that has been yielding positive results for individuals with Pierre Robin sequence (PRS), directly addressing craniofacial correction, aiming for improved respiratory quality, and enabling adequate mandibular elongation. This study aims to evaluate mandibular distraction osteogenesis and its efficacy in patients with PRS. The literature search encompassed PubMed, Medical Literature Analysis and Retrieval System Online, Latin American and Caribbean Health Sciences Literature, and the Scientific Electronic Library Online, using the DeCS descriptors: "Pierre Robin Syndrome" AND "Distraction Osteogenesis" AND "Micrognathism". Studies have demonstrated the efficacy of MDO as a therapeutic approach to correcting respiratory and feeding problems, with the FEMOD protocol showing promise in improving upper airways and correcting micrognathia. Although studies point to MDO as a good solution for treating mandibular retrognathia in PRS patients, further research is still needed to validate results, compare different treatment approaches, assess long-term surgery effects, and identify potential late complications. This underscores the importance of a multidisciplinary approach to treating these patients.