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In contrast to epidermoid cysts of the dermis, epidermoid cysts of the kidneys are exceptional. This report explains an uncommon patient with renal epidermoid cyst who presented with flank pain and previous history of renal stones. Our patient underwent excisional resection. The histopathological evaluation discovered cyst wall covering by squamous epithelium and containing keratinous materials compatible with the epidermoid cyst. Based on pathology findings, the patient didn’t receive any more treatment. Because pathology evaluation is necessary for the diagnosis or rules out the possibility of malignancy, biopsy before surgery is advised.