Obstructive sleep apnea (OSA) is common in children with achondroplasia and yet there is no consensus on the most effective treatment for this sleep disorder in these children. The purpose of this review is to provide an update on the most used interventions and their effectiveness Results: A total of 149 children with obstructive sleep apnea and achondroplasia were studied and outcomes for both surgical and non-surgical interventions reported. The most commonly performed intervention, in 60 children, was adenotonsillectomy (T&A), with a decrease in AHI from 14.2 to 7.2. Adenoidectomy without tonsillectomy was performed in 7 children with 5 having residual OSA and additional surgeries. Cervicomedullary decompression was performed in 15 children with a decrease in AHI from 58 to 38. Two children had a tracheostomy. Midface distraction was described as an alternative to tracheostomy, that also improved CPAP therapy. The primary non-surgical option was CPAP therapy. For milder OSA, nasal steroids were an option. Conclusion: Adenotonsillectomy was the most commonly performed surgical intervention and had the lowest rate of persistent OSA and the best outcome. Children who underwent adenoidectomy alone frequently had persistent OSA and a subsequent tonsillectomy but were generally younger. Cervicomedullary decompression is used to treat neurologic symptoms but can improve but not resolve OSA. Midfacial advancement may be considered in children as an alternative to tracheostomy and improve CPAP therapy. Children with mild to moderate OSA, can be treated with nasal steroids/PAP or could be closely observed if not candidates for surgery.