Mucoepidermoid carcinoma of the gastrointestinal tract is a rare entity. The present case is the first report of mucoepidermoid carcinoma in the large bowel. A 71-year-old man was admitted to our hospital to undergo surgery for a recurrent chordoma of the hip. A barium enema and colonoscopy were carried out to assess whether there was any relationship between the recurrent chordoma and the rectum. An ulcerating tumor was thus identified in the ascending colon. A radical right hemicolectomy was carried out. In the resected specimen a 3-cm ulcerating lesion was observed. The tumor consisted of PAS-positive mucin-producing cells, epidermoid cells, and intermediate cells. No differentiated squamous cell carcinoma cells were identified in any part of the tumor. The malignant cells proliferated mainly in the submucosa but also invaded the muscularis and serosa. The tumor was diagnosed as a mucoepidermoid carcinoma of the ascending colon. Unfortunately, despite chemotherapy, the patient developed liver metastases and died of liver failure 10 months postoperatively.