In this manuscript for the first time we describe the concomitant diagnosis of primary renal non-Hodgkin lymphoma (PRL) and of a papillary urothelial cancer in a patient with megaloblastic anemia. PRL is a rare disease, since the kidney is one of the extranodal organs usually not containing lymphoid tissue. The disease usually affects adults with an average age of 60 years and slight male preponderance. Flank pain is the most common presenting symptom and different histologies have been reported. A review of literature indicated that simultaneous diagnosis of PRL and papillary urothelial carcinoma of the urether, makes our case unique. The early diagnosis of both diseases allowed the eradication of the two neoplasms by nephro-ureterecthomy and by performing subsequent systemic chemotherapy.