In the context of ARVC, a systematic review of the validation of the ARVC risk score can provide insights into the accuracy and reliability of this score in identifying patients at high risk of ARVC. Digital databases were searched to identify the relevant studies using Medical Subject Headings (MeSH). A total of 8 studies were included in this systematic review. A total of 8 studies were included in this review. The review found that the sensitivity of the ARVC risk scores ranged from 80 to 95%, and the specificity ranged from 31 to 79%. The PPV was 55%, and the NPV was 88%. The ARVC score provided a C-index for a 5-year VA risk prediction of 0.84 [95% CI (0.74–0.93)] and a Harrell C-index of 0.70 (95% CI 0.65–0.75). The calibration slope was 1.01 (95% CI 0.99–1.03). ARVC score demonstrated a significant event 5-year threshold between 15 and 20% and the classical ARVC 5-years/freedom-from-VA rate was 0.76(0.66–0.89) and the non-classical form 5-years/freedom-from-VA rate was 0.58 (0.43–0.78). In conclusion, the validation of ARVC risk scores is an essential step toward improving the accuracy of ARVC diagnosis and risk stratification. Further studies are needed to establish the accuracy and reliability of ARVC risk scores and to address the limitations of the current evidence.