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Cushing's syndrome has been believed to be a paraneoplastic syndrome of renal cell carcinomas. However, there appears to be a dearth of compelling evidence to substantiate this notion. The only eligible documentation of Cushing's syndrome due to ectopic adrenocorticotropic hormone secretion by renal cancer in English literature dates back to 1988, and it pertains to a deceased patient discovered during an autopsy. Here, we present the first case of Cushing's syndrome as a paraneoplastic feature of renal cancer which showed complete resolution following surgical removal of the tumor. Additionally, we conduct review of the literature on this particular subject.