A 22-year-old man had been given a diagnosis of idiopathic portal hypertension in childhood. In June 2001, a hepatic nodule which gradually increased in size over the next 2 years, was detected in the left hepatic lobe. In February 2003, a left lateral segmentectomy was performed. Histological examination of the nodules suggested focal nodular hyperplasia (FNH)-like hyperplasia. Computerized tomography performed 2 years later showed other hepatic nodules, and a liver biopsy was performed. Histopathological examination conducted at this time also suggested FNH-like hyperplasia. Owing to the substantial enlargement of the nodules and frequent recurrence, it can be difficult to distinguish between benign and malignant lesions.