Lymphomatoid granulomatosis is a rare form of EBV related large B-cell lymphoma, with an angiocentric and angiodestructive growth pattern in extranodal sites. Brain involvement is relatively frequently found, but not as the initial and unique clinical manifestation. When it occurs, brain stroke is the most frequent clinical picture. Moreover, in that setting, patient can die without a definitive diagnosis, and autopsy is then required for a definitive diagnosis purpose.