Abstract: Objective　To study the clinical characteristics of the temperature instability in Prader-Willi syndrome (PWS). Methods　The clinical characteristics of one case of PWS with recurrent fever were retrospectively analyzed. Results　 A 5-month-old boy who had such clinical characteristics as low central muscle tension, sucking weakness, feeding difficulties, cryptorchidism and white skin was diagnosed as PWS by chromosomal microarray analysis and methylation-specific PCR. In the course of the disease, the infant presented recurrent fever. The pathogenic bacteria culture and virus antibody detection showed negative, and the treatment of reducing fever, anti-infection and immune support were ineffective. Conclusions　The cause of the recurrent fever is unknown and central fever is suspected. Whether the temperature instability is the clinical feature of PWS still needs a lot of clinical researches.