BACKGROUND Reactive lymphoid hyperplasia (RLH) of the liver is an uncommon benign lesion. It is usually difficult to differentiate from hepatocellular carcinoma (HCC), peripheral-type cholangiocellular carcinoma (CCC), combined HCC-CCC, and liver metastases. On account of its rarity and controversial issues, we report a case report and review the literature to discuss its clinical features, treatments, radiological, and immunohistochemical characteristics. METHODS A 54-year-old woman had a history of primary biliary cirrhosis and chronic cholecystitis. She complained of finding a lesion in the right liver during her last medical check-up by abdominal B type ultrasound. The Contrast-enhanced computed tomography revealed a circular and low-density lesion in the right posterior lobe of the liver, approximately 22.0 × 18.7 mm in size. On magnetic resonance imaging, the lesion showed low-signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Laboratory test results were almost normal. METHODS After the postoperative pathological and immunohistochemical examination, the patient was finally diagnosed as having RLH. METHODS The patient received right posterior lobe hepatectomy and cholecystectomy. RESULTS The patient was discharged 11 days after surgery. No evidence of recurrence was noted 1 year after the surgery. CONCLUSIONS Although RLH of the liver is rare, it is necessary to be considered in a liver lesion, especially in female patients. This case report may advance the understanding of RLH of the liver and reduce the number of mistakenly diagnosed patients.