Dentinogenesis imperfecta is one of the morphological changes in tooth structure that can occur during the toothcells differentiation. Prognosis of dentinogenesis imperfecta usually is not favorable, considering the great difficulty to protect and restore the affected teeth, due to low receptivity to bonding restorative materials. This case report aims to draw the dentistïs attention to the characteristics of this anomaly, which is difficult to treat and leaves serious sequelaes. Early diagnosis is fundamental to a better prognosis. A 5 years old malepatient presenting deciduous teeth with vitrified aspect and brownish-yellow color, enamel wear, obliteration of the pulp chamber and areas of periapical radiolucency. The diagnosis of dentinogenesis imperfecta type II was realized and clinical, radiographic and histological aspects were documented. Dentists should be able to realize an early diagnosis to favor a better prognostics for the patient and minimize the complications related to the anomaly.