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Background:   Papillary tumors of the pineal region are rare neuroepithelial lesions that were described for the  1st  time in the WHO 2007 classification. Management of such lesions remains controversial.    Case Description:   We describe the case of a 26-year-old female who presented with intracranial hypertension syndrome secondary to a 1.9 cm 3  lesion of the pineal region causing hydrocephalus. The patient benefited from an endoscopic third ventriculocisternostomy and a biopsy of her lesion in favor of a papillary tumor of the pineal region. After discussion of the surgical risks, the patient refused the surgical option and a stereotactic radiosurgery (SRS) was performed. She improved both clinically (allowing her to regain autonomy) and radiologically (reduction of 60% of tumor volume) at 1 year follow-up.    Conclusion:   Because of the rarity of the lesion, literature is yet not able to find consensus concerning management of such lesion, but SRS has proven efficiency for these Grades II or III lesions with high recurrence rates. Therefore, it should be considered as a primary therapeutic option allowing good outcome with low risks for the patient.

Papillary tumor of the pineal region: Is stereotactic radiosurgery efficient for this rare entity?