Objective: To investigate the clinicopathologic features, diagnosis and treatment as well as prognosis of primary non-Hodgkin’s lymphoma (NHL) of the common bile duct. Methods: The data of a patient with primary NHL of the common bile duct treated in the Affiliated Tumor Hospital of Guangxi Medical University, and together with the data of 34 cases of primary NHL of the common bile duct reported from 1982 to 2017 at home and abroad were analyzed. Results: The patient was an 81-year-old male, who was diagnosed as primary NHL (diffuse large B cell lymphoma) of the common bile duct by postoperative pathological findings after undergoing pancreatoduodenectomy. No adjuvant anti-tumor therapy such as systemic chemotherapy and regional radiotherapy was performed after operation, and no recurrence was noted 4 months later. Of this patient plus the reported 34 patients, 21 cases (60.0%) were males and 14 cases (40.0%) were females; the age at onset ranged from 4 to 81 years, with a median age of 57 years; the clinical manifestations included jaundice (88.6%) and abdominal pain (40.0%), enlarged superficial lymph nodes (11.4%) and concomitant fever (11.4%); imaging manifestations in some cases showed space occupying lesion in the common bile duct or thickening and stenosis of the common bile duct wall; four cases underwent systemic chemotherapy, 10 cases underwent surgical resection, 16 cases underwent surgical resection combined with systemic chemotherapy, 1 case underwent surgical resection combined with local radiotherapy, 3 cases underwent surgical resection combined with systemic chemotherapy and local radiotherapy, and treatment regimen in 1 case was unknown. The majority of cases had a passable prognosis after treatment, and one case survived more than 72 months; there were no significant differences in survival times between patients undergoing surgical resection alone and those undergoing chemotherapy alone or other combined treatments (all P>0.05). Conclusion: Primary NHL of the common bile duct is a rare condition in clinical practice, lacks typical clinical manifestations, and the laboratory tests and imaging examinations have no obvious specificity. So, its clinical diagnosis is difficult, and definite diagnosis depends on postoperative pathology and immunohistochemistry. Selection of appropriate treatment options is critical for improving the survival of the patients.