Familial glioblastoma multiforme is a rather uncommon entity, being in most 
cases associated to known genetic disorders (as Turcot syndrome, Li-Fraumeni 
syndrome, neurofibromatosis, etc.). However, familial gliomas have also been 
described, although less frequently, independently of these genetic syndromes 
showing some special features regarding its etiology and clinical 
manifestations. Less than 10% of gliomas may be considered as true multicentric 
tumours either synchronous or metachronous in clinical presentation. 
Metachronous glioblastomas have been associated to better prognosis in some 
studies, with genetic studies having found clear differences among the tumors 
within same patients. Familial glioblastoma with metachronous presentation is an 
exceptional disorder. These tumors show special therapeutic implications due to 
the limitations of radiotherapy once the patient has already irradiated. A 
variety of non-specific mutations have been found in these patients but true 
characterization of this disorder remains unclear and will be based on further 
genetic studies. We present a clinical report on a patient harbouring a familial 
and metachronous glioblastoma. The main aspects of this entity are reviewed.