Abstract: Objective To introduce a 15-day-old male newborn who was hospitalized in the Guangdong Women and Children Hospital due to repeated fever,and the newborn was finally diagnosed as congenital ectodermal dysplasia. Method 15-day-old male hospitalized newborn with dry skin, desquamation, sparse hair, absent sweating and recurrent fever were diagnosed by skin biopsy. Result Skin biopsy showed that the skin appendage disappeared and it was diagnosed as congenital ectodermal dysplasia. Congenital ectodermal dysplasia is a rare genetic disease that can affect the development or function of teeth, hair and sweat glands. The most common symptom is recurrent fever. Conclusion After excluding infection and other factors causing fever, for newborns with recurrent fever of unknown causes,the possibility of congenital ectodermal dysplasia should be considered to avoid delaying the disease.