Nodular regenerative hyperplasia of the liver (NRHL) is a chronic non-sclerotic liver disease characterized by hepatocellular nodule formation with mild fibrosis. The etiology is unclear and may be related to oral contraceptive use and autoimmune diseases. Pathologically, NRHL is associated with an abnormal distribution of intrahepatic blood flow, which is manifested by hepatocyte atrophy in areas of reduced blood flow and compensatory proliferation in normal areas to form regenerative nodules. Clinical manifestations include asymptomatic early stage and portal hypertension, abdominal distension, abdominal pain, and gastrointestinal bleeding in the middle and late stages. Imaging shows that diffuse NRHL manifests as small regenerative nodules widely distributed in the liver with a diameter of less than 3 mm, while focal NRHL manifests as larger isolated nodules. Ultrasound usually shows hypoechoic or isoechoic nodules; CT scan shows hypointense or isointense nodules, and enhancement scan shows delayed-phase central enhancement with a peripheral hypointense ring; MRI may show a “donut” sign in the hepatobiliary phase. The diagnosis of NRHL should be combined with clinical manifestations and pathological examination and should be differentiated from hepatic adenoma and focal nodular hyperplasia of the liver.