Down syndrome (DS) is an autosomal chromosomal anomaly resulting from trisomy of all or a critical part of chromosome 21. It affects approximately 1 in 700 to 800 live births. Despite the development of prenatal diagnosis, the incidence of DS births is predicted to remain static or even to increase over the next decade, partly due to increased maternal age in Western societies. Many of the medical and physiological characteristics of DS have direct consequences for the oral health of subjects affected and indirect consequences for the quality of life of persons with DS and their carers. This article aims to give an overview of the current literature concerning the orofacial problems confronting patients with DS, and to explain the different treatment modalities available.