Ectodermal dysplasias are a heterogeneous group of disorders characterized by developmental dystrophies of ectodermal structures. These tissues include the skin, hair, nails, eccrine glands and teeth.1 Hypohydrotic Ectodermal Dysplasia is the most common type and is usually inherited as an X-linked recessive trait. It is characterised by the triad of signs which comprises of sparse hair (atrichosis or hypotrichosis), abnormal or missing teeth (anodontia or hypodontia), and inability to sweat due to lack of sweat glands (anhidrosis or hypohidrosis). The incidence of ectodermal dysplasia in males is estimated at 1 in 100,000 births.2 The aim of this case report is to examine a rare and unusual case of familial hypohidrotic ectodermal dysplasia, study the signs and symptoms of this condition and review the diagnostic and treatment modalities to be followed.