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Renal cell carcinoma (RCC) is known as the internist tumor because of the myriad paraneoplastic manifestations associated with it. One of the rarely described paraneoplastic manifestations associated with this malignancy is hyperglycemia. Only 11 cases in the English and Japanese literature have been reported. We report the occurrence of paraneoplastic hyperglycemia with a rare variant: mucinous tubular and spindle cell variant of RCC. To the best of our knowledge, the association of paraneoplastic hyperglycemia with this variant has not been reported earlier.