BackgroundCardiac tuberculosis is rare and usually manifests as tuberculous pericarditis. Involvement of other part of the heart is unusual and descriptions in the pediatric literature are confined to few case reports regarding mainly myocardial tuberculosis.Case presentationWe describe a case of pulmonary miliary tuberculosis associated with intracardiac left atrial tuberculoma in an immunocompetent eleven-month-old infant successfully treated with surgery and antituberculous therapy.ConclusionAlthough unusual, involvement of endocardium in disseminated tuberculosis should be kept in mind.