In two men aged 19 and 24 years, a rare malignant intracranial germ cell tumour was diagnosed in the pineal gland region and in the second patient in a suprasellar position as well. Although histological confirmation is required in general to diagnose a malignancy, obtaining it can be difficult and potentially dangerous in case of intracranial neoplasms. The clinical picture of a young patient with an intracerebral tumour localised in the midline of the brain and increased levels of the tumour markers alpha-foetoprotein and/or human chorion gonadotrophin (beta-HCG) in blood and/or CSF makes any other diagnosis highly unlikely. There is no place for radical surgery in the first-line treatment of malignant intracerebral germ cell tumours because of the sensitivity to radio- and chemotherapy. Also, the sensitivity to chemotherapy makes it possible to reduce radiation volume and dose in an effort to avoid the serious complications of craniospinal irradiation. Both patients responded well to chemotherapy based on cisplatin followed by radiotherapy.