Catheter-related arterial thrombosis (CAT) is increasingly recognized in children. Available data are scarce and based on expert opinions. This systematic review aimed to identify knowledge on paediatric CAT. Among 3,484 publications, 22 met inclusion criteria. Fourteen reported on CAT due to umbilical arterial catheter (UAC), two to extremity indwelling catheter (EIC), one to both and five to cardiac catheter (CC). The overall cumulative incidence of CAT was 21% (95% confidence interval [CI], 13-31) with a relative incidence of 20% (95% CI, 10-33) for UAC and 11% (95% CI, 3-21) for CC-related CAT. The incidence of EIC-related CAT ranged from 3.4 to 63%. Clinical presentation of CAT included symptoms of acute limb ischaemia (79%, 95% CI, 54-97), arterial hypertension (55%, 95% CI, 23-86) and congestive heart failure (28%, 95% CI, 7-53). Underlying conditions of UAC-related CAT included prematurity (70%, 95% CI, 31-98), respiratory distress syndrome (56%, 95% CI, 46-65), asphyxia (41%, 95% CI, 15-69), infection (32%, 95% CI, 13-55), persistent ductus arteriosus (28%, 95% CI, 13-45), meconium aspiration (16%, 95% CI, 8-25) and congenital heart disease (9%, 95% CI, 2-19). Congenital heart disease was the likely condition in EIC- and CC-related CAT. Antithrombotic treatment included thrombolysis (71%, 95% CI, 47-91), heparin (70%, 95% CI, 41-94) and thrombectomy (46%, 95% CI, 10-95) alone or in combination. Complete resolution rate of CAT was 82% (95% CI, 65-96). Long-term complications included arterial hypertension (26%, 95% CI, 0-66) and limb amputation (12%, 95% CI, 1-31). The overall all-cause mortality rate was 7% (95% CI, 2-14). In conclusion, CAT occurs at an increased incidence in neonates and children and is potentially associated with poor outcome. However, limited data are available on paediatric CAT. This systematic review identifies the rationale for further studies on CAT in paediatric patients.