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Introduction: Dentinogenesis imperfecta is an autosomal dominant hereditary disease originated in the stage of histodiferenciation during the odontogenesis, it´s a form of a localized mesodermic dysplasia, characterized by alteration of dentinal proteins. Objective: To show of treatment of dentinogénesis imperfecta in children with the utilization of stainless steel crowns and composite. Case report: An 8 year old girl affected by the disease came to the dental clinic because of her parents concern. The girl suffers from pain during the ingestion of cold and sweet foods, change in the form and color of the teeth and also psychological alteration regarding her personal appearance. A clinical and radiographic exam was performed. It showed a mix dentition with brownish discoloration of all the teeth and loss of dental tissues especially in the first permanent molars because of that a loss of vertical dimension was observed. The x rays showed presence of dental follicles of all the permanent teeth and bones structures with a normal appearance. After the recollection of familial history, a genetic consultation, clinical and radiographic exam, was performed and the dentinogenesis imperfect was diagnosed. A stainless steel crows were the alternative of treatment in the first permanent molars and the second temporary molars with the intention of restore the vertical dimension and to decrease the dentinal sensibility, the anterior teeth were also restored with Composite. Principal Comment: It’s vital to make a certain diagnose and an early treatment of this disease to avoid bigger tissues destruction. In children the treatment with stainless steel crowns and composite is a good alternative until the adulthood and others prosthetic treatment can be done.