BackgroundThe aim of this study was to assess in amyloidosis prevalence in Korea between 2006 and 2015.MethodsPrimary diagnoses related to amyloidosis, regardless of subtype, were collected from the Korean National Health Insurance Service from 2006 through 2015.ResultsOverall, the age-standardized prevalence of amyloidosis was 0.93 (95% confidence interval (CI) 0.81, 1.04) persons per 100,000 persons in 2006 and 1.91 (95% CI 1.78, 2.05) persons per 100,000 persons in 2015. This included an increase from 0.43 (95% CI 0.35, 0.51) to 1.04 (95% CI 0.94, 1.14) persons per 100,000 persons in men and from 0.49 (95% CI 0.40, 0.57) to 0.87 (95% CI 0.77, 0.96) persons per 100,000 persons in women. In particular, the age-standardized prevalence of amyloidosis showed a greater increase in patients aged 65 years or older and in patients aged 45–64 years than in patients aged 20–44 years, for both men and women.ConclusionsThe overall age-standardized prevalence of amyloidosis was approximately 2 persons per 100,000 persons in 2015. The overall age-standardized prevalence of amyloidosis increased between 2006 and 2015, especially in individuals aged 45–64 and older than 65 years.Electronic supplementary materialThe online version of this article (10.1186/s13023-017-0705-2) contains supplementary material, which is available to authorized users.