Amelogenesis Imperfecta is a hereditary anomaly that affects the enamel of human teeth and is not associated with any systemic disorder of affected patients. The affected teeth are disturbed in coloration, thickness and resistance. The rehabilitation of amelogenesis imperfecta in a child must take into account the development of the child's teeth, the health of the periodontal tissues and the maxillary and mandibular growth. This article reports the endodontic and occlusal rehabilitation of a 14-year-old girl affected with autosomal recessive hypocalcified type of amelogenesis imperfecta with multiple periapical cysts.