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Exophytic primary gastric squamous cell carcinoma and Primary gastric squamous cell carcinoma (SCC) is rare, and the simultaneous Helicobacter pylori infection has not been reported in the literature. Here, we presented a patient with concurrent H. pylori gastritis and primary gastric SCC. A 54-year-old Hispanic man presented with diarrhoea, chills, night sweats and weight loss of 16 lbs for the previous 6 weeks. Abdominal CT revealed large exophytic mass from the stomach infiltrating multiple organs. Biopsy was performed and histology showed squamoid features. Immunohistochemistry stain was positive for p40, CK5/6, CK7 and Helicobacter type organisms. Patient was diagnosed with primary gastric SCC and has been receiving chemotherapy. We also reviewed the diagnosis, prognosis and treatment of primary gastric SCC.