Very few reports are available on the occurrence of abnormality of both enamel and dentin. This case has some characteristiced of both amelogenesis imperfecta and dentinogenesis imperfecta. Clinically, the enamel of primary dentition was completely absent and when the permanent teeth came to the pediatric dept. of Dankook University Dental Hopital for treatment. Fixed-removable type resin plate was delivered to increase vertical dimension and to solve esthetic and functional defects. Hypoplastic teeth were restored either stainless steel crown or composite resin restoration or both. The periodic recall check and oral hygiene education are recommended.