A 50-year-old man with Whipple's disease was found to have a positive Kussmaul's sign, prominent jugular V waves and a pansystolic murmur. Echocardiography revealed severe tricuspid regurgitation and moderate aortic and mitral regurgitation. Thickening of the noncoronary aortic cusp, calcification of the mitral annulus and a possible calcified density embedded in the left ventricular free wall were also noted. A review of the literature demonstrates high prevalence of cardiac manifestations in patients with Whipple's disease. Constrictive pericarditis, valvular deformity, myocarditis, coronary arteritis, congestive heart failure and sudden death have been documented. No clear relationship between the extent of cardiac involvement and the severity of the systemic disease has been shown, and the efficacy of antibiotic treatment for cardiac lesions remains to be demonstrated.