Diagnosis and treatment of tumours and vascular malformations in the region of the pineal gland continue to challenge the neurosurgeon's skill. Due to vital vascular and brain structures in the region, microsurgical removal of such masses is often impossible. During the past nine years, we have managed 47 patients with pineal region mass lesions using stereotactic techniques for diagnosis and treatment. In order to determine further therapeutic options, 15 patients underwent stereotactic biopsy of pineal region tumours. In all patients, the histologic diagnosis obtained served to direct further therapy. Thirty-two patients were treated with stereotactic radio-surgery for pineal region tumours or vascular malformations. During the follow-up period, one patient underwent delayed microsurgical resection of a midbrain angiographically occult vascular malformation. No other patient required microsurgical intervention after a stereotactic procedure. In all 47 patients, no significant morbidity or mortality occurred after stereotactic biopsy or radiosurgery. Empiric treatment of pineal region tumours with fractionated radiation therapy is no longer warranted. Image-guided stereotactic technology provides a safe method to accurately diagnose and effectively treat selected pineal region masses. After definitive histologic diagnosis is established, proper treatment may be instituted. Options for treatment include stereotactic radiosurgery for selected tumours and vascular malformations, microsurgical resection of benign tumours or fractionated external beam radiation therapy for malignant germ cell and glial tumours. Stereotactic surgery should be the first option in the diagnosis and therapy of pineal region masses.