An otherwise healthy 14-year-old girl presented with polycythemia and elevated serum erythropoietin. A lesion was identified in the liver by ultrasound scan and computerized axial tomography. The pathology findings of the mass excised by hepatic segmentectomy showed focal nodular hyperplasia that stained positively for erythropoietin by immunohistochemistry. Resection resulted in a cure of the polycythemia with serum erythropoietin, hemoglobin, and hematocrit returning to normal range in subsequent follow-up. To our knowledge, this is the first report of focal nodular hyperplasia resulting in secondary polycythemia.