Background. Several studies have revealed that congenital missing in primary dentition is uncommon and the most frequently affected teeth are lateral incisors, usually accompanied with the missing of succedaneous teeth. The congenital absence of primary canines is especially a rare finding in the dental literature. Case report. This case report presented a 3-year-8-months-old Chinese girl with her multiple caries, was found congenitally missing of maxillary bilateral primary canines and mandibular right primary canine. However, the succedaneous teeth of the missing primary canines were all evident on the panoramic radiograph, and all of the permanent tooth germs except for the third molars were developing consistent with her chronological age. Conclusion. There are only a few studies in the literature have reported the finding of missing primary canines. The aim of this case report is to increase the awareness that congenital missing of primary canines does happen despite of the low incidence, and provide future therapeutic directions of the management in dealing with the complications in primary and mixed dentition.