Pre-eruptive intracoronal resorption (PIR) is characterized by radiolucent lesion within coronal dental tissue and rarely seen in unerupted permanent teeth. The affected teeth are generally asymptomatic and detected during routine dental radiographic examination. Amelogenesis imperfecta (AI) is a group of relatively rare inherited abnormalities in enamel formation and/or calcification. A history of consanguineous marriage should be considered in individuals with the type of autosomal recessive AI (ARAI). Several dental alterations especially intracoronal resorption of unerupted teeth and failure or delayed eruption are more common in AI patients than in unaffected individuals. This report presents the clinical and radiographic findings of ARAI and PIR cases simultaneously seen in three generations of a family and oral rehabilitation of an unusual member of the same family.