This report presents a case of 69-year-old woman, who was operated due to renal tumor. Apart from renal neoplasm, the adjacent adrenal gland contained another one tumor in medulla of the organ. The renal lesion was diagnosed renal cell carcinoma, clear cell type with undifferentiated cell sarcomatoid component. The adrenal neoplasm was composed of wavy S100-positive, Schwann-like cells and dispersed chromogranin A-reactive ganglion cells to be consistent with mature ganglioneuroma. It was accompanied by coexistent myelolipoma that contained hematopoietic cells including clearly visible megakaryocytes and foci of fat. To our knowledge, our paper is the first to report sporadic clear cell renal cell carcinoma with sarcomatoid cell type component and mature adrenal ganglioneuroma with myelolipoma in the same patient.