We present the first report on familial pineocytoma. The propositus, a 31-year-old man, presented with incontinence due to a cystic and haemorrhagic tumour of the pineal region. His 34-year-old sister, who had suffered from tinnitus for several years, also had a pineal tumour. Histopathology following tumour resection revealed pineocytomas (WHO grade 1). Cerebral MRI examinations of the patient's brother and father did not reveal any pineal region abnormalities. Their mother had developed breast cancer at the age of 43. Although not impossible, it is rather unlikely that pineocytomas occurring in siblings are pure coincidence because of the rarity of this type of tumour.