OBJECTIVES:  TTR-FAP is an irreversible, fatal, and rare autosomal dominant genetic disease that has been identified in >30 countries. The global ATTR-FAP prevalence is difficult to determine, in part, due to under-diagnosis. The present analysis was conducted to provide global prevalence estimates of diagnosed ATTR-FAP based on a synthesis of available prevalence information. METHODS:  A comprehensive systematic search of electronic databases was conducted, supplemented with manual review of abstracts presented at key clinical conferences, grey literature, and personal communications/files. Reports published between 2005 and 2015 were included, with older key references included on a case-by-case basis. For countries with published prevalence estimates, the total number of persons with ATTR-FAP was calculated as the product of prevalence and population size. Extracted prevalence estimates were extrapolated to countries without reported prevalence estimates, but where TTR-FAP cases have been reported. RESULTS:  28 regional or national prevalence estimates were identified in 13 countries. Using this information, 4,859 (range, 4,366 to 11,459) ATTR-FAP cases were projected from 12 of the 13 countries in which prevalence estimates exist (Spain was excluded as only a regional estimate was available). If other countries (n=22, excluding India/China), where cases are known to exist but no prevalence estimates are available, are included, the projected prevalence is estimated at 5,961 (range, 4,600 to 17,259) cases. Including China/India, the projected prevalence is 9,908 (range, 5,441 to 38,042). CONCLUSIONS: