Rationale: Primary hepatic mucosa-associated lymphoid tissue (MALT) lymphoma is an extremely rare disease. To the best of our knowledge, only 67 cases had been reported in 39 English literatures to date. The aim of this study was to add a new case of this disease to the literature and to review the current literature. Patient concerns: A 50-year-old man was incidentally identified with a solitary mass of 5 cmin diameter in the left lobe of the liver. Diagnoses: Based on the results of imaging studies, intrahepatic cholangiocellular carcinoma was suspected, and then surgery was performed. Microscopic findings showed that the tumor was a hepatic MALT lymphoma, and immunohistochemical analysis revealed that the lymphoma cells were CD20+, CD79a+, BCL-2+, CD3-, and CD5-. Interventions: The patient received rituximab after surgery. Outcomes: He was free of disease for 13 months at the time of this report. Lessons: Since previously published case reports and our case described nonspecific clinical features of this rare disease, it was usually misdiagnosed before histological confirmation and surgery resection may be a good choice for both diagnosis and local therapy.