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A 31-year-old woman with a 5-year history of Crohn's disease was admitted to our hospital because of recurrent right lower quadrant pain and diarrhea. Abdominal computed tomography showed multiple fistulas between the terminal ileum, the sigmoid colon, and the cecum, and mucosal wall thickenings due to an active inflammatory process and mucosal enhancements. Colonoscopic examinations showed a finger-like projection of a polypoid mass at the ileocecal valve, long-neck, finger-like pseudopolyps at the cecum, and soft, lumen narrowing and multiple pseudopolyps at the sigmoid colon and the intact rectum. There was healing scarring of the anal fistula. These findings were compatible with those of Crohn's disease. Histologic findings were chronic inflammation with erosion and regenerative crypt epithelium. The patient underwent infliximab therapy. She underwent a right colectomy, a Hartman's procedure, and a small bowel segmental resection due to multiple fistulas. The pathologic diagnosis was a signet-ring-cell carcinoma with non-caseating granuloma in the ascending colon, ileum, and sigmoid colon. We report this case of Crohn's disease associated with a colonic signet-ring cell carcinoma.