Objective To investigate the level of family management in children with Prader-Willi syndrome （PWS） and analyze its influencing factors, aiming to provide reference for targeted family intervention.Methods A total of 172 parents of children with PWS were investigated with the Family Manageme Measure, the Family Hardiness Index and the Perceived Social Support Scale.Results In the Family Management Measure, the dimension scores of parental mutuality, child′s daily life, condition management ability, view of condition impact, family life difficulty, and condition management effort were （28.11±7.38）, （14.10±3.46）,（38.92±5.17）,（36.05±4.48）,（51.69±8.95）, and （16.63±2.69）, respectively.Regression analysis showed that, children′s age, time to diagnosis, nutritional stage, abnormal behavior, average per capita monthly income, family resilience and perceived social support were the influencing factors of family management （all P＜0.05）.Conclusion Parents of children with PWS encounter more difficulties in daily life care and disease management.Attention should be paid to those families of children with older age, late diagnosis, and abnormal behaviors, and those families with low income, poor resilience and social support.