Tumors of the pineal region represent a diverse collection of tumors with a variety of natural histories. This diversity necessitates accurate histologic diagnosis to allow rational therapeutic planning. Evaluation of a pineal lesion should begin with craniospinal MRI and analysis of the cerebrospinal fluid (CSF). Whereas certainty of the histologic diagnosis is now a requirement for treatment in Western nations, some Asian centers continue to recommend a test dose of radiation therapy based on the high incidence of germinoma in those countries. If there is high clinical suspicion of a germinoma or tectal glioma, stereotactic or endoscopic biopsy may be pursued. All other lesions should be referred for open biopsy with microsurgical techniques. This approach provides adequate tissue for diagnosis, may be curative in low-grade tumors, and may substantially improve survival in patients with malignant tumors. If open surgery is not desired by the patient or practitioner, stereotactic or endoscopic biopsy may be followed by radiosurgery for localized, well-demarcated tumors. Radiation therapy is the first-line therapy for germinomas. Although the optimal radiation dosage and volume have not been decided, the current Children's Oncology Group trial may offer definitive evidence to address this dilemma in germ cell tumors. Evidence of CSF seeding requires craniospinal radiation and adjuvant chemotherapy regardless of tumor type. Diagnosis of any of the malignant tumors (non-germ cell tumors, pineoblastomas, and parenchymal tumors of intermediate determination) also requires craniospinal radiation (with local tumor doses of at least 50 Gy) and adjuvant chemotherapy (generally platinum based). Patients with tectal gliomas may undergo excision with or without postoperative radiation; however, they also may be observed with vigilant follow-up alone.