Ectodermal dysplasia (ED) is a rare genetic disorder that is brought on by abnormalities in the development of tissues, organs, and other accessory appendages formed from embryonic ectoderm. Ectodermal dysplasia usually presents with Bilateral congenital tooth absence. Other ectodermal abnormalities such as anhidrosis, asteatosis, hypotrichosis, and salivary gland anomalies may coexist with anodontia or hypodontia. This case report highlights the unique features when compared with a typical Ectodermal Dysplasia