Ectodermal dysplasia is a heterogeneous group of syndromes, that involves the abnormalities of ectodermal derivatives like skin, hair, nails, salivary glands and teeth. The hall mark of the syndrome involves a triad of signs, comprising of atrichosis or hypotrichosis, anodontia or hypodontia, and anhidrosis or hypohidrosis . In addition, dystrophy of nails and palmoplantar hyperkeratosis is often present. Here is a case report of 9 year old boy with ectodermal dysplasia, associated with dental problems and its management.