Objective: The simultaneous association of gastric carcinoma with gastric lymphoma is a rare event. Method: We describe a 52 years old man, who had been diagnosed as marginal zone lymphoma in a cervical lymph node biopsy 3 years previously.No endoscopic examination was evaluated then. There was no pathological appearance in the gastrointestinal system by CT scanning and patient was considered nodal marginal zone lymphoma. Patient had taken 8 cycles of chemotherapy which consisted of rituximab, cyclophosphamide, vincristine, and prednisolone.2,5 years after completion of chemotherapy, a gastric wall thickening compatible with gastric lymphoma or linitis plastica was detected on CT and gastroscopic examination was indicated. Endoscopic examination revealed an erosive, fragile, hemorrhagic, malignant-looking lesion with a diameter of 4.5 cm in the cardia. Besides, rugae of fundus and corpus were severely rough and hyperemic suggesting a diffuse malignant infiltration. Multiple biopsies were taken from cardia, fundus and corpus of the stomach. Results: Cardiac samples showed pancytokeratin positive signet ring cells which contain neutral and acidic mucine in microscopic examination. Samples of corpus and fundus did not show similar carcinoma cells, instead, there was diffuse infiltration of atipical lymphocytic infiltration consistent with marginal cell lymphoma both morphologically and immunophenotypically. Conclusion: Co-occurrence of carcinoma and marginal zone lymphome is a rare event and dramatically exacerbates prognosis of a patient with such an indolent lymphoma.