Objective To study the treatment status of phenylketonuria (PKU) children,and analyze the factors influencing phenylketonuria childre's treatment compliance in Xinjiang area. Methods Totally 33 PKU children who were screened and delayed to be treated in the Neonatal Screening Center of the First Affiliated Hospital of Xinjiang Medical University from January 2011 to January 2015 were enrolled in this study.On the basis of the time of diagnosis,they were divided into the new screening group (14 cases) and delayed group (19 cases),the PKU children's treatment status was investigated by reading medical records and telephone follow up,univariate and multivariate Logistic regression analysis and χ² test were used to analyze the factors influencing PKU children's treatment compliance in Xinjiang area. Results The family income below average level (OR=2.815,P<0.05),and the national minority(Uyghur,Hui,Kazak) (OR:1.332~2.240,P<0.05)were risk factors influencing PKU children's treatment compliance.The PKU children's treatment compliance of new screening was higher than that delayed to be treated (P<0.05).There was no significance difference in treatment compliance between PKU children with different genders (P>0.05). Conclusions The family income(below average level),the national minority(Uyghur,Hui,Kazak)and the time of diagnosis (delayed to be treated) are important factors influencing PKU children's treatment compliance.Expanding the coverage of neonatal disease screening and equipping with a doctor of ethnic minorities for ethnic minority patients,is one way to improve the treatment compliance.