Dentinogenesis imperfecta (DGI) is an inherited dentin disease resulting in fragile teeth that affect the primary and permanent dentitions. It causes physical and aesthetic problems, including tooth discoloration and staining, and is responsible for great damage and wear to the entire dentition. DGI is classified into three types, with COL1A1 and COL1A2 gene mutations present in the first type, and mutations of the DSPP gene present in the second and third types. Treatment is focused on the restoration and replacement of damaged and worn teeth, as well as aesthetic improvements. Restorative, prosthodontic, and orthodontic treatment is often necessary. The aim of this paper is to shed light on this pathological disease and the importance of obtaining an early diagnosis which is necessary to implement treatment.