SETTING: Tuberculosis (TB) related stigma is associated with lack of treatment adherence. Individual perceptions of stigma differ by societal context. Limited data are available on variations of TB stigma worldwide. OBJECTIVE: To describe the influence of TB stigma on knowledge, attitudes and responses to TB and to identify similarities and differences across countries. DESIGN: Systematic review of international descriptive studies. RESULTS: A total of 1268 studies were identified from PubMed/Medline, Web of Science, Cochrane, PsycINFO and Cumulative Index to Nursing and Allied Health Literature database searches. Eighty-three studies from 35 countries met the inclusion criteria for English, peer-reviewed, original and non-interventional studies. Variation and similarities in the influence of TB stigma on knowledge, attitudes and responses to TB across countries were identified. Stigma antecedents included negative attitudes and misperceptions regarding the causes of TB and the association with the human immunodeficiency virus. Decisions about illness disclosure and choices between traditional healers and public or private providers were influenced by TB stigma. Sex-influenced perceptions and management of TB and public health responses contributed to TB stigma. CONCLUSION: Our findings confirm cultural variations with respect to TB and the potential for stigma. Cultural variations should be considered in the development of interventions aimed at reducing stigma and improving treatment adherence.