A 26-year-old man with primitive neuroectodermal tumour (PNET) is reported. The tumour originated from the cervical spinal cord and was resected partially. Few months later dissemination of the tumour to the meninges occurred. Familial history revealed that the first daughter of the patient had died in age of 14 months three years earlier of a tumour of the right cerebral hemisphere, also diagnosed as PNET.