Clinical history and morbid anatomy findings of a new case of ataxia-telangiectasia are reported. A 26 years old man, with overt signs of the disease since the age of 8, died for decompensated cirrhosis; in the last year he suffered for cerebral haemorrhage; post-mortem examination showed venous angiectasias in cerebral white matter that caused two macroscopical areas of haemorrhagic infarction and multiple pin-pointed haemorrhages. We suggest that longer survival of patients with A.T., due to better management of infectious complications, permits the appearance of vascular abnormality not only in skin and mucosae, but also in deep organs, generally protected from environmental damages.