[Objective] To report a rare case of non-Hodgkin’s lymphoma originating from the ureter and to review the literature. [Methods] To summarize the diagnosis and treatment of this patient in the context of this case and to analyze the relevant domestic and international literature. [Results] Lymphoma originating in the ureter is rare, with only three cases reported so far in China and nearly 20 cases reported abroad. Most of the patients were middle-aged and elderly men, and clinical symptoms were mainly urinary tract obstruction, hydronephrosis, hematuria and renal discomfort. The prognosis correlated with the malignancy of the pathology. [Conclusion] Patients with lymphoma originating in the ureter usually have partial or complete ureteral obstruction as the main symptom at the initial diagnosis, and surgical treatment to relieve the obstruction is recommended as soon as possible. Systematic examination should be performed to determine the clinical stage, and treatment principles should be established according to the pathological type and clinical stage.