ABSTRACT
Here we report two cases of anhidrotic ectodermal dysplasia in a family presented to us with intermittent fever, developmental delay, frontal bossing, hypohydrosis, sparse hair and oligodontia. Ectodermal dysplasias are a large hereditary group of disorders which are usually manifested as X-linked recessive hypohidrotic ectodermal dysplasia (HED) and has a full expression in males, whereas females show little to no signs of the disorder. Ectodermal dysplasia are group of rare genetic disorders characterized by faulty development of ectodermal structures and thought to be due to embryonic defect in ectodermal development. Patients frequently consult dentists for delay in tooth eruption so the appropriate awareness of the disease among dentists is essential for early diagnosis.