Down syndrome or trisomy 21 is the most common chromosomal aberration in humans that causes intellectual disabilities ranging from mild to severe. In addition to intellectual disabilities, people with Down syndrome face numerous diseases of various organ systems, and the biggest problems occur in the form of heart anomalies. Due to impaired immune responses and abnormalities in the structure of the respiratory system, they are prone to respiratory infections with an increased risk of developing sepsis. Vision, hearing and speech problems are very common. Children with Down syndrome have higher incidence of malignancies, primarily leukemia. Among other diseases, there are disorders of the digestive and endocrine systems, orthopedic problems and neurological diseases - autism and epilepsy. The characteristics of the craniofacial area of children with Down syndrome are hypoplastic maxilla and the entire base of the skull, small and protruded mandible and dental anomalies, primarily hypodontia and microdontia. Angle's class III is present, often accompanied by other orthodontic anomalies. The tongue is hypotonic and protruded with lip incompetence. Periodontal disease is very common and it can occur around the age of 11, while caries is not so frequent. In the dental care of children with DS, problems occur in terms of poor motivation of parents and patients to maintain oral health, but also the lack of experience of dentists in working with these patients. In therapy, the emphasis is on eliminating infection from the oral cavity as it can affect general health. It should be taken into account that children with DS are often the high-risk patients with possibility of developing infectious endocarditis.