Introduction: AL amyloidosis is widely regarded as a rare disease, but characterization of its epidemiology has scarcely been reported. The purpose of this systematic review is to estimate the incidence rate of AL amyloidosis and examine population differences. Methods: MEDLINE, PubMed, and Google Scholar were searched from their inception until November 13, 2021 using search terms AL amyloidosis or immunoglobulin light-chain amyloidosis or light-chain amyloidosis or primary amyloidosis and incidence or epidemiology. Random-effects meta analysis of all cohort studies reporting an incidence rates for AL amyloidosis was performed. The quality of each study was assessed using a modified Newcastle-Ottawa scale. Subgroup analysis was performed based on geographical region. Results: Six studies with data from 2502 diagnosed cases of AL amyloidosis from 5 countries were included. The pooled incidence rate for AL amyloidosis was 10.48 per one-million person years (95% CI, 8.99 to 11.96). There was moderate heterogeneity in the data, which was eliminated with a subgroup analysis according to geographical region. AL amyloidosis was found to be more common in the Americas (incidence rate 11.52 per one-million person years, 95% CI 11.04 to 12.00) than in the Europe (9.10 per one-million person years, 95% CI, 7.62 to 10.58). Conclusion: There is a low worldwide incidence rate for AL amyloidosis, supporting the characterization of AL amyloidosis as a rare disease. The incidence of AL amyloidosis appears to vary in different populations, which suggests further well-designed studies are needed to elucidate underlying etiological factors and better inform clinical suspicion for AL amyloidosis.