Cardiac involvement in Whipple's disease is well established. However, clinical consequences beside antibiotic therapy have rarely been reported. Our observation of a middle-aged man with increasing dyspnea, fatigue, chest pain, and dizziness leading to admission to a cardiology department demonstrates that cardiac symptoms may represent the main symptoms in patients with Whipple's disease. The diagnosis was not made prior to upper endoscopy, performed because of diarrhea, and revealed Whipple's agent now classified as Tropheryma whippelii, which is a PAS-positive rod-shaped bacterium in the macrophages of the intestinal lamina propria. The aortic valve was replaced after the intestinal symptoms were resolved by antibiotic treatment reducing the number of infectious agents in the duodenal mucosa. Histological analysis of the aortic valve demonstrated the presence of PAS-positive rod shaped material as the most likely cause of aortic insufficiency. Five months after valve replacement, the patient had completely recovered from intestinal and cardiac symptoms. Still under antibiotic treatment 16 months later, no more PAS-positive macrophages were detectable in the intestinal mucosa.