Introduction: Ectodermal dysplasia is a rare, non-progressive, genetic disorder resulting from abnormal development of two or more tissues at a time which are derived from the embryonic ectoderm. It classically manifests with skin, eccrine gland, nail and hair changes, with an incidence of 1 in 1, 00,000 births. More than 170 different syndromes have been identified. Xlinked recessive anhidrotic dysplasia (XLHED) being the most common type, which is expressed in males. Case Report: A six-year-old boy, was bought to the pediatric outpatient department with the complaints of fever on and off since month months. The parents gave history of recurrent episode of hyperpyrexia, with heat intolerance, absent sweating and delayed dentition in the past. On examination the child had peculiar facies, characterized by malar hypoplasia, flattening of nasal bridge, everted lips, wrinkled periorbital skin, pegged shaped tooth, low set ears, scanty, hypopigmented hair on the head; with absent eyebrows and eye lashes. Even after thorough evaluation and investigation, no focus of infection was found, ectodermal dysplasia was then considered and a skin biopsy done, which showed absence of skin appendageal structures. Conclusion: We report a rare case of anhidrotic ectodermal dysplasia in a 6-year-old boy, thus emphasizing the need for considering EDA as a differential diagnosis for neonates and infants with history of fever of unknown origin specially when associated with delayed dentition and hypotrichosis. An early diagnosis would have prevented unnecessary antibiotic misuse.