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Cor triatriatum dexter (CTD) is a rare congenital heart disorder that is result of persistence of the right valve of the embryonic sinus venosus. Clinical significance of CTD is related to degree of obstruction. Asymptomatic patients may be diagnosed during echocardiographic examination performed for unrelated reasons. Presently described is case of a 12-year-old girl with initial diagnosis of acute rheumatic fever whose echocardiogram revealed presence of CTD. Aim of this report was to emphasize that diagnosis of CTD can be difficult and requires careful evaluation of the right atrium by echocardiogram.