Lymphomatoid granulomatosis (LG) is an uncommon lymphoproliferative disease characterised by angiocentric and angioinvasive cellular infiltrates. The lung is the usual primary site with secondary central nervous system (CNS) involvement in 20% of cases. Primary cerebral LG is a rare but potentially treatable disease with protean manifestations. Diagnosis is problematical and may be delayed when extracerebral disease is absent or occult. Six cases of cerebral LG are presented, and the clinical features, laboratory investigations, neuroimaging and pathological findings are reviewed.