Objective To summarize the diagnosis and treatment experiences of focal nodular hyperplasia (FNH) . Methods The clinical data of 48 patients with FNH who were admitted to the Xiangya Hospital from February 2010 to August 2014 were retrospectively analyzed. All the patients received complete serologic tests, abdomen B ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) examinations. The pathological examinations were done on the patients undergoing surgical treatment. The surgical treatment was done on the unconfirmed FNH patients according to location of space-occupying lesions. The conservative treatment was done on the confirmed FNH patients without clinical symptoms. All the patients were followed up via outpatient examination and telephone interview up to September 2014, and the follow-up included the history review, clinical symptoms and medical examinations. Results Forty-seven patients had normal liver function and 1 had high level of total bilirubin (TBil) . The serum tumor markers including AFP, CEA and CA19-9 in all the patients were negative. Fifty-five foci were detected by imaging examination, 41 foci were single mass and 7 foci were multifocality. Forty-eight patients were definitively unconfirmed with FNH by B ultrasound. The results of CT examinations in 38 patients showed as follows: low density was in 31 patients and isodensity in 7 patients, including striped and radiated low density showing in the center of tumor in 20 patients and 3 patients with the confirmed FNH. The results of MRI examinations in 10 patients showed as follows: 3 patients were confirmed as with FNH; all the 10 patients showed rather homogeneous enhancement in arterial phase; 5 patients were accompanied with radiated foci in the center of tumor, and showed high signal on T2WI, no enhancement in early arterial phase and enhancement in delayed arterial phase. The central scar showed low signal on T1WI and high signal on T2WI. Two patients were confirmed as with FNH by percutaneous trans-hepatic histopathological examinations. Forty patients without the confirmed FNH underwent surgical treatment, including 31 undergoing open hepatectomy and 9 undergoing laparoscopic hepatectomy. No patients had cirrhosis of liver. During operation, 47 foci were detected, 16 of which located at the left lobe of liver and 31 of which located at the right lobe of liver. Local tumor resection of liver was carried out on 30 patients, right hemihepatectomy or extended right hepatectomy on 7 patients, left hemihepatectomy on 3 patients and combined treatment of cholecystectomy on 12 patients. The operation time, volume of blood loss and diameter of foci were (78-255) minutes, (80-720) mL and (4. 6 ±1. 6) cm (range, 1. 5-11. 5 cm) . Forty patients were confirmed as with FNH by pathological examination, and 8 patients were cured by conservative treatment. All the 48 patients were followed up for 21 months (range, 1-56 months) and survived well without recurrence and complications. Conclusions The clinical symptoms of FNH are atypical and unobvious. The preoperative enhanced CT and MRI examinations can help improve the diagnosis and differential diagnosis of FNH. The surgical treatment could be performed on the patients with significant clinical symptoms and without definitive diagnosis, and the patients with definitive diagnosis should be treated by conservative treatment with the good prognosis. Key words: Focal nodular hyperplasia; Hepatectomy; Diagnosis; Therapy