A systematic literature review of cystic echinoccocosis (CE) frequency and symptoms was conducted. Studies without denominators, original data, or using one serological test were excluded. Random-effect log-binomial models were run for CE frequency and proportion of reported symptoms where appropriate. A total of 45 and 25 articles on CE frequency and symptoms met all inclusion criteria. Prevalence of CE ranged from 1% to 7% in community-based studies and incidence rates ranged from 0 to 32 cases per 100,000 in hospital-based studies. The CE prevalence was higher in females (Prevalence Proportion Ratio: 1.35 [95% Bayesian Credible Interval: 1.16-1.53]) and increased with age. The most common manifestations of hepatic and pulmonary CE were abdominal pain (57.3% [95% confidence interval [CI]: 37.3-76.1%]) and cough (51.3% [95% CI: 35.7-66.7%]), respectively. The results are limited by the small number of unbiased studies. Nonetheless, the age/gender prevalence differences could be used to inform future models of CE burden.