The clinical and neuroradiological features of focal midbrain tumors in 12 children are described, and the results of their surgical management are presented. Patients with a focal midbrain tumor usually exhibit either symptoms and signs of raised intracranial pressure caused by an obstructive hydrocephalus (50%) or symptoms and signs caused by pressure on the tegmentum and cerebral peduncles. The lesions are confined to the tectal plate or tegmentum with possible extension upward to the thalamus and downward to the pons, displacing but not invading these structures. The edges of the tumor are well defined, and the large majority have a solid consistency with intense regular enhancement after intravenous contrast. Radical resection is hardly ever feasible in brain stem tumors, but in this series, significant reduction of the tumor mass was obtained in 75% of the patients, with no surgical mortality and minimal surgical morbidity and with the majority of patients showing clinical improvement postoperatively. All tumors were nonpilocytic, low-grade astrocytomas. Six patients received adjunctive radiotherapy. The mean follow-up period is 2.5 years, and all patients are alive and doing well. We conclude that focal midbrain tumors in children appear to be a distinct subgroup of brain stem tumors and are very amenable to surgical resection with an excellent long-term prognosis.