Pineal region tumors represent 0.4% to 1.0% of intracranial tumors in American literature. Obtaining a tissue diagnosis is the cornerstone of the rational management of pineal lesions. The initial surgical decision involves choosing between a stereotactic biopsy and open microsurgical procedures. Open resection facilitates the maximal removal of tumor volume and has diagnostic accuracy and improved prognosis. Stereotactic biopsy is less invasive and has a lower risk of complications. A review of all major series reporting stereotactic biopsy for pineal region lesions reveals a mean diagnostic yield of 94%, with a morbidity of 1.3% and a mortality of 8.1%.