Pineal cavernous angioma is a vascular malformation that has a prevalence lower than 1%. The etiology is debated. It is believed that it is originated from an autosomal dominant inheritance or from radiotherapeutic treatment. Complete resection enables the cure and prevents complications of the natural evolution of the lesion, mainly hemorrhagic events. A female patient, with 67 years of age, presented mental confusion and visual acuity deficit, which evolved to periods of psychomotor agitation. A magnetic resonance imaging (MRI) scan of the head showed a lobulated mass lesion in the pineal region with hypersignal on T1 and hyposignal on the susceptibility weighted imaging (SWI) sequence. Hydrocephalus was also noticed. The patient underwent a microsurgery for complete lesion resection though a supracerebellar/ supratentorial access. The anatomopathology revealed an arteriovenous malformation compatible with cavernous angioma. The pineal cavernous angioma is a rare malformation that should be considered in the differential diagnosis of expansive lesions of the pineal gland.