Objective To investigate the relationship between the polymorphism of repeatable sequence of DRD4 exonⅢ 48 bp gene and depressive symptoms in children with Gilles de la Tourette syndrome (TS). Methods A total of 112 children who were diagnosed as TS according to the criteria of the Fourth Edition of Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) (the TS group) and 71 healthy children (the control group) were selected. The polymorphous points of DRD4 gene were genotyped by the amplification refractory mutation system (ARMS) polymerase chain reaction (PCR) genotyping assay method and the depressive levels of children with TS were measured by the children's depression inventory (CDI). Results Among 112 children with TS, 29 (25.9%) were diagnosed with depression. The differences of genotype frequency and allele frequency of DRD4 exonⅢ 48 bp VNTR among the TS group, comorbid depression group, and control group were not statistically significant (P>0.05). The differences of CDI scores and factor scores between 61 children who carried long repeat allele group and 51 children who carried short repeat allele group were not statistically significant (P>0.05). Conclusion The DRD4 exonⅢ 48 bp VNTR may not correlate to the TS comorbid depression.