Focal nodular hyperplasia is a rare hepatic tumor in children representing only 2% of all pediatric hepatic tumors. We report a case in teen-ager who had a symptomatic portal cavernoma at 9 years of age, and had been treated by porto-mesenteric surgical shunt. At 16 years, liver ultrasounds revealed multiple hepatic nodules. Focal nodular hyperplasia was suggested by CT scan and magnetic resonance imaging. Given the symptoms and the unusual and multiple lesions, diagnosis was confirmed by surgical biopsy. FNH pathogeny is discussed in this context.