We report a case of a 58-year-old man with a history of long standing familial Mediterranean fever (FMF) and AA amyloidosis, who developed renal papillary carcinoma and renal pelvic urothelial carcinoma simultaneously. Although the association between chronic inflammatory states like FMF and AA amyloidosis has been well established, the relationship between amyloidosis and solid tumors is not defined as clearly. Furthermore, to the best of our knowledge, co-existence of two different types of kidney malignancy with amyloidosis in a patient with FMF has not been reported. Our patient was admitted to hospital with gross hematuria and renal insufficiency. Imaging studies revealed mass lesions in the middle portion of the right kidney. Right radical nephrectomy showed extensive amyloid deposition, co-existing with renal papillary carcinoma and poorly differentiated invasive urothelial carcinoma.