Objective: To describe a rare syndrome among children and to urge pediatricians to consider in considering such diagnosis when investigating fever of unknown etiology among neonates. Methods: Case report of Anhidrotic Ectodermal Dysplasia Syndrome in a ten day old newborn hospitalized in a NICU at Hospital Geral de Caxias do Sul. The child presented recurrent episodes of fever since the firsts days of life. Results: The patient presented dry mucous, dry skin and fever. Skin biopsy was performed in his back region. A lack of sweat and sebaceous glands as well as hypoplasia of follicular structures were identified. The patient is being monitored at the clinic, receiving supportive treatment. Conclusion: Anhidrotic ectodermal dysplasia syndrome is a rare disorder that must be considered when investigating newborns with recurrent episodes of fever. During the neonatal period, the clinical manifestations of the disease are subtle and unspecific. Such findings become more visible after a few months of life. There is no definitive treatment for this syndrome.