Tumors in the pineal region usually present with acute hydrocephalus. Histologic diagnosis is most important, as it greatly influences treatment, because over 17 tumor types occur in this area. Biopsies of these lesions play an important role in further management. Pineocytomas are benign and rare tumors that typically exhibit a slow progression. However, the appropriate treatment for pineocytoma varies. Surgical excision was considered for good long-term outcomes; however, this may not always be possible. Radiotherapy also appears to be effective in patients with residual pineocytomas. We report a case of pineocytoma with hemorrhagic transformation and complicated hydrocephalus. The patient refused to undergo aggressive tumor excision. Thus, the patient only underwent endoscopic biopsy and external ventricular drain (EVD) implantation, but the outcome was acceptable. In addition, we reviewed the current management strategies for pineocytomas in the literature.