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Colon cancer of Peutz-Jeghers syndrome with gallolyticus endocarditis. We report a case of Peutz-Jeghers syndrome with gallolyticus endocarditis which has not yet been reported. Colon cancer was observed and implicated in Peutz-Jeghers syndrome. A 44-year-old female with fever and heart murmur was diagnosed as infective endocarditis caused by streptococcus gallolyticus. After treatment with antibiotics and mitral valbuloplasty, we performed gastrointestinal endoscopic studies and found polyps in stomach and colon. Histological findings of a large pedunculated colon polyp revealed hamartomatous polyp with a lesion of adenocarcinoma with adenoma. She had pigmentation of digits. Her father had also digits pigmentation and died of pancreas cancer. Peutz-Jeghers syndrome with colon cancer was incidentally diagnosed by infective endocarditis and subsequent colonoscopy.