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Aggressive advanced gastric cancer in a patient with autosomal dominant polycystic kidney disease. A 60-year-old man with autosomal dominant polycystic kidney disease presented with malaise, melena, and epigastric discomfort. Esophagogastroduodenoscopy revealed a massive elevated gastric cancer lesion involving the cardia. Histopathological evaluation of a biopsy specimen showed poorly differentiated adenocarcinoma. Fluorodeoxyglucose-positron emission tomography revealed significant fluorodeoxyglucose uptake in the stomach, liver, bones, and bone marrow. He was diagnosed with metastatic gastric cancer resistant to chemotherapy, and he developed bone marrow carcinomatosis and disseminated intravascular coagulation and died 8 weeks after disease onset. A statistically significant association is reported between autosomal dominant polycystic kidney disease and gastric cancer. Moreover, the specific clinical features observed in our patient could be attributed to the molecular disorders like PC-1 and mechanistic target of rapamycin that are known to occur in autosomal dominant polycystic kidney disease.