Down syndrome (DS) was first described by John Langdon Down (1866) at mid-nineteenth century and one century later, the DS primary cause due to trisomy 21 was reported (Lejeune et al., 1959). DS is predominantly due to non-disjunction of chromosome 21. It is not a disease; however the affected individuals have a greater risk in acquiring many systemic and oral conditions. DS is the most common autosomal chromosomal anomaly with an incidence of 1 in 600 to 1000 live births in all races and economic groups. The extra genetic material in these patients causes a delay in the way a child develops, either mentally or physically. The present article discusses a case report of a 5-year old male patient with the classical features of Down syndrome. The skeletal and soft tissue features, aberrations in dental, periodontal and caries characteristics are discussed and dental management of such patients.