Background & Objective: Varicella is a common infection during childhood and generally self-limiting. However, it can rarely cause neurological complications. Isolated acute peripheral facial palsy (APFP) is extremely rare during primary varicella infection with estimated incidence of <0.01%. There have also been conflicting opinions on its natural history, prognosis and management worldwide. We aimed to establish the natural history, prognosis and treatment for varicella-related isolated APFP in immunocompetent individuals, without co-morbids. Methods: Systematic review was performed with systematic literature search in Google Scholar and PubMed. Data was analysed with statistical analysis software. Results: Thirty cases were included. The complete remission rate of APFP was 66.67% for nontreatment group and 72.22% for treatment group (p=0.643). Early and late treatment group had a similar complete remission rate of 88.89% and 80% respectively (p=1.000). However, early treatment group (within 3 days of onset) had achieved complete remission 3 weeks earlier than the late treatment group (p=0.091). Antiviral group tends to have better outcome than steroid monotherapy group, although statistically insignificant (p=0.055). Conclusions: This condition generally has good prognosis even without treatment. However, early treatment and antiviral therapy may at least accelerate remission and reduce morbidities although these cannot alter the final outcome. Clinicians may consider antiviral therapy if patients present within 3 days of onset. These findings need to be applied with caution, considering the limitations of our review.