Nonoperative management of focal nodular hyperplasia (FNH) is an accepted paradigm in adults, but current management strategies for children vary substantially between institutions. We reviewed medical records at Johns Hopkins Hospital between January 1, 1998, and December 31, 2008, to investigate the diagnosis, treatment, and outcome of pediatric patients with a pathologic diagnosis of FNH to provide additional data to help formulate management guidelines for this disease. Ten pediatric patients were identified as having a pathologic diagnosis of FNH, either by biopsy sample (n = 5) or hepatic resection (n = 5). The mean age of the patients was 12.1 years, and most were female (n = 7). Mean tumor size was 5.7 cm (range, 0.8-13 cm). Four of 5 patients whose FNH was diagnosed by biopsy alone developed no sequelae, and 1 patient eventually required surgery for mass effect. Patients with either large lesions (≥5 cm) or symptoms were referred for resection. Observational management of small lesions that can be confidently diagnosed as FNH appears to be safe and appropriate. Surgical resection should be reserved for large or symptomatic lesions amenable to resection.