Synchronous cancers in patients with esophageal cancer are rare. We present a female nonsmoker with gastrointestinal symptoms that led to the diagnosis of HER-2/neu positive esophageal adenocarcinoma. During the staging workup, she was found to have a synchronous early-stage clear cell renal carcinoma. The patient underwent an esophagogastrectomy and radical nephrectomy and remains disease-free at a 12-month follow-up visit. We offer a detailed literature review on esophageal cancer and second primary malignancies seen in this context. Our case is unique as the literature suggests more advanced staging at diagnosis and poorer prognosis in persons with synchronous or metachronous cancers of esophagus and kidney. We believe that studies involving more patients are needed for the long-term prognosis assessment in these patients.
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