Trisomy 18 is characterized by: psychomotor disabilities, dysmorphic features, organ malformations, including mental retardation, growth deficiency, poor motor ability, micrognathia, microcephaly, congenital heart defects, and kidney abnormalities. The oral findings typically observed in these patients are: cleft lip and a high, narrow, and sometimes cleft palate. The degree of severity of the malformations is directly related to life expectancy. Only 5% to 10% of affected infants survive beyond the first year of life. Although trisomy 18 has been widely investigated from a medical standpoint, there is a lack of reports addressing the oral manifestations and dental treatment approach in affected children, presumably due to their shortened life expectancy. The purpose of this article was to present the case of an 8-year-old child diagnosed with trisomy 18 and address the clinical features observed--emphasizing the disease-specific oral, craniofacial, and dental findings. Dental care management of the patient is described.