We studied the frequency and pattern of tooth agenesis in a Danish population with Down syndrome, trisomy 21 (46 females and 54 males). The control group consisted of a normal Danish population (2424 females and 2431 males) [Rølling, 1980: Scand J Dent Res 88:365-369; Ravn and Nielsen, 1973: Tandlaaegebladet 77:12-22]. We found that individuals with Down syndrome have an occurrence of agenesis that is some 10 times greater that in the general population with a higher frequency in males than in females. Agenesis occurred more frequently in the mandible than in the maxilla and most often on the left side. The highly significant differences were primarily found in the occurrence of agenesis of the mandibular central incisors, followed by the maxillary lateral incisors and second premolars and the mandibular second premolars. The main components in the pattern of agenesis observed in Down syndrome are supposed to be related to the peripheral nervous system and abnormal cartilagenous tissue. The present study on Down syndrome suggests that the dentition, with its many different anomalies, from agenesis to malformation, can be used as an indicator in evaluating different aspects in the patheogenetic of aneuploidy conditions.