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Abstract Primary lung signet‐ring cell carcinomas are a rare entity and only a few cases of pure signet‐ring cell carcinomas of the lung are reported in the English literature. They usually have an aggressive behavior and a poor prognosis because in most cases they are identified at an advanced stage. We present a unique case of primary signet‐ring cell carcinoma of the lung because the patient was HIV positive, a heavy smoker, and also the tumor, discovered incidentally during chest x‐ray, was a pure type of signet‐ring cell carcinoma. Surgical therapy associated with chemoradiotherapy represents the gold standard in the care of these patients.