Amelogenesis imperfecta (AI) is a hereditary condition that affects the clinical features and structure of the enamel. The enamel formation diseases are inherited and might be X-linked, autosomal dominant, recessive, sex-related, or sporadic. Dental professionals should evaluate such patients completely, both clinically and radiographically, to detect any cysts associated with impacted or unerupted teeth. This report describes the case of a 10-year-old patient affected by amelogenesis imperfecta with a dentigerous cyst involving an impacted tooth and an unerupted tooth of the maxilla.