A cross-sectional study was conducted in children with tic disorder (TD) in western China who were recruited at a pediatric neurology clinic. We used the Pediatric Quality of Life Inventory TM Version 4.0 Short Form Generic Core Scale (PedsQL 4.0) and the eight-item Morisky Medication Adherence Scale to assess the QOL and medication adherence, respectively. A multiple linear regression model was used to examine the factors affecting QOL. In total, 198 patients participated in the study, and the response rate was 93.4%. The mean age of the participants was 7.73 ± 2.60 years, and 77.3% (153/198) of the participants were male. The mean overall score of the PedsQL 4.0 Generic Core Scales was 82.25 ± 11.68. The highest mean score was for physical functioning (92.49 ± 12.85), whereas the lowest mean score was for school functioning (67.93 ± 22.09). The mean scores for emotional functioning and social functioning were 75.20 ± 17.70 and 87.22 ± 14.13, respectively. PedsQL scores were positively correlated with lower Yale Global Tic Severity Scale (YGTSS) scores, the absence of comorbidity, and higher adherence scores. The score of school functioning and emotional functioning is not high in children with TD in western China. In conclusion, as medication adherence is associated with quality of life, it is necessary to develop a multidisciplinary approach to improve medication adherence among children with TD.