Dental agenesis may be present in an isolated familiar manner, or occur as a part of a syndrome.To date, this clinical trait seems to have been overlooked in patients with Crouzon syndrome.The aim of the present study was to investigate dental agenesis and dental agenesis patterns in a population of persons with Crouzon syndrome in Sweden. Serial panoramic radiographs of 26 individuals with Crouzon syndrome (20 males, 6 females) were examined.Third molars were excluded from the assessment. The prevalence of agenesis for at least one tooth was 42.3%. Each affected patient was found to have up to 5 missing teeth. Upper and lower second premolars were the most frequently congenitally missing teeth. Eleven dental agenesis patterns of the entire dentition were identified, as described by the tooth agenesis code (TAC). All patterns were unique and asymmetric,with only one exception, a symmetric pattern of the maxillary and mandibular second premolars. In conclusion, persons with Crouzon syndrome were found to have a high prevalence of dental agenesis and a remarkable variability of dental agenesis patterns. It is important to be aware of this clinical situation, especially when orthodontic treatment planning for these patients is performed as early as in the mixed dentition.