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We describe a rare case of primary composite tumor with bipartite differentiation of the esophagogastric junction. An 88-year-old Japanese man with a history of distal subtotal gastrectomy was admitted to Harima Hospital of Ishikawajima-harima Heavy Industries with dysphagia of 2 months duration. Endoscopy showed a round, sharply demarcated lesion with a smooth surface in the lower esophagus. Distally, the lesion was irregular and elevated with a deep central depression. The gastric cardia was slightly elevated with a granular mucosa. After surgical resection, histological and histochemical findings showed a primary composite tumor with bipartite differentiation of the esophagogastric junction. There is no evidence of recurrence for 21 months after surgery without adjuvant chemotherapy. The present case indicates the necessity for a careful evaluation for accessory lesions in composite tumors containing small cell carcinoma of the esophagogastric junction, and that resectional surgery might not only be the treatment of choice, but also important in the evaluation of immature composite tumor without distant metastases. [ABSTRACT FROM AUTHOR]