We report a case of benign schwannoma presenting as a rapidly enlarging liver mass in a 67-year-old man and review the literature on nerve sheath tumors of the hepatobiliary system. Such tumors, which may occur in patients with and without neurofibromatosis, are very uncommon in the liver. The case reported herein, to our knowledge, is the first well-documented benign schwannoma of the liver occurring in a patient without neurofibromatosis.