Paragangliomas are rare neoplasms arising from undifferentiated cells of the primitive neural crest. We report a case of a 34-year-old patient with renal paraganglioma. Complete surgical resection of the tumor was performed without intraoperative incident. The diagnosis was made based on immunohistochemical and ultramicroscopic analyses. The postoperative course was uneventful. Based on the obtained results, we discuss the clinical aspects of the case and the diagnosis.