(1) Introduction: Tourette syndrome (TS) and chronic tic disorder (CTD) are common neurodevelopmental/-psychiatric disorders. The aetiological factors that contribute to the pathogenesis of TS/CTD are still poorly understood. The possible risk factors for TS/CTD are considered to be a combination of genetic, immunological, psychological and environmental factors. A comprehensive systematic review was conducted to assess the association between aetiological factors and TS/CTD. (2) Methods: Electronic databases, including PubMed, Embase, Web of Science, Wanfang data, and CNKI, were searched to identify the etiological factors of children and adolescents (≤ 18 years) with TS/CTD based on a case-control study. Quality assessments were performed according to the Newcastle-Ottawa scale (NOS). (3) Results: According to sample sizes and NOS values, recent evidence may support that genetic factors (BTBD9 and AADAC), immunological factors (streptococcus and mycoplasma pneumoniae infections), environmental factors (conflict, history of perinatal diseases, and family history of neurological and psychiatric diseases and recurrent respiratory infections) and psychological factors (major life events) are associated with the pathogenesis of TS/CTD. (4) Conclusions: Some risk factors in different categories may be the etiological factors of TS/CTD, but there is a lack of studies on the interaction among the factors, which may require more attention in the future.