Objective: Kashin-Beck disease (KBD) is an endemic deformable bone and joint disease, which seriously affects the quality of life (QOL) of patients. We tried to conduct a cross-sectional study of the QOL of KBD patients by a new KBD quality of life (KBDQOL) questionnaire.Methods: 252 KBD patients came from Northwest China, and 260 healthy people living in the same area as KBD patients served as the control group. KBDQOL questionnaire was used to evaluate the QOL of all objects.Results: The average score of physical functions, activity limitations, support of society, mental health and general health of KBD patients was significantly lower than that in control group. There was no statistical difference in economics between KBD patients and control group. The univariate analysis showed that age, height, weight status, education level and grade of KBD had a statistically significant effect on KBDQOL score. The results of multivariate analysis showed that grade of KBD was the influencing factor of physical function score; sex, age, height, grade of KBD and duration of symptoms were the influencing factors of activity restriction score; grade of KBD was the influencing factor of economic score, age and grade of KBD were influencing factors of general health.Conclusions: The QOL of patients with KBD was significantly lower than that of healthy people living in the same place. The KBDQOL questionnaire may be a promising tool for assessing the QOL of KBD patients.