BACKGROUND: Although nonoperative management is an accepted practice for most adults with focal nodular hyperplasia (FNH), questions remain about the safety and feasibility of this strategy in children. Our aim was to review the clinical features of children with FNH and determine current management patterns. METHODS: We reviewed records of all children and adolescents with FNH managed at our institution from 1999 to 2009 and performed a MEDLINE search to identify all published cases of FNH in the pediatric population. RESULTS: A total of 172 patients with FNH were identified, including 11 at our institution. The median age at diagnosis was 8.7 years and 66% were female. Median tumor size was 6 cm, and 25% had multiple lesions. Thirty-six percent were symptomatic at presentation. Twenty-four percent had a history of malignancy. Management included resection (61%), biopsy followed by observation (21%), and observation alone (18%). Indications for resection included symptoms (48%), inability to rule out malignancy (24%), tumor growth (15%), and biopsy-proven concurrent malignancy (9%). CONCLUSIONS: Although FNH is a benign lesion that is typically managed nonoperatively in adults, most children with FNH currently undergo resection because of symptoms, increasing size, or inability to confidently rule out malignancy.