Ectodermal dysplasias is a rare hereditary disorder that results due to disturbances in the ectoderm of the developing embryo. The classic features that define this condition are partial or complete tooth agenesis which is a congenital lack of one or more teeth of primary and or permanent teeth, sparse hair, dystrophies of nails and lack of sweat glands. Since the pediatric dentists are the first to be consulted in case of loss of teeth, this article is a sincere effort to spread awareness about this rare entity. A case of eleven year old girl child is reported hereby who presented with a complaint of missing teeth, dry skin and brittle nails.