Hereditary transthyretin amyloidosis (ATTRv), caused by TTR gene mutations, affects various tissues, with ocular involvement in about 10% of patients.<h4>Objective</h4>To describe and quantify ocular manifestations in ATTRv through a systematic review.<h4>Methods</h4>We reviewed studies on ocular manifestations of ATTRv, including only articles in Portuguese and English, excluding case reports, conference abstracts, and duplicates.<h4>Results</h4>Sixteen studies with 1792 patients were analyzed. This evidence concerns the gene TTR and Familial transthyretin-related amyloidosis.