Dentinogenesis imperfecta is an phenotypic alteration in the formation of the organic matrix. It causes the rapid and progressive wear of tooth structure, which may compromise tooth function and aesthetics. This is a case of a 1y, 8m-old child with dentinogenesis imperfecta. All teeth presented with an opalescent appearance and grayish color hue. Compromised structural integrity was noted as excessive wear and fracturing of the enamel from the dentin layer. With low doses of midazolam (oral) and chloral hydrate (rectal) administration, in a hospital environment, sedation was used to aid full mouth rehabilitation. Direct bonded restorations were performed on primary maxillary incisors and indirect restorations, pre-made on a plaster model using composite resin, were performed on primary mandibular incisors. After 32 months, we observed that diagnosis and early treatment allowed preventive maintenance of the patient's primary dentition, maintaining tooth function and esthetics.