In patients with pineal tumors, clinical symptoms are due to direct compression of adjacent structures. The most common signs include increased intracranial pressure (80%) caused by obstruction of the sylvian aqueduct, and Parinaud syndrome (50%) caused by direct compression of the superior colliculi. Hearing loss is rare in patients with tumors in this location. The authors report on the case of a 12-year-old boy in whom a malignant pineal germ cell tumor was found together with the unusual occurrence of severe hearing loss due to direct bilateral compression of the inferior colliculi. This condition resolved completely after tumor regression.