To study the clinico-pathological characteristics and differential diagnosis of granulomatous meningioencephalitis caused by acanthamoeba.The clinical features, CT scan findings and post mortem pathological changes of 3 patients afflicted with granulomatous amoebic encephalitis (GAE) were analysed.GAE is a chronic clinical entity. Clinical manifestations include headache, low fever, seizures, hemiparesis and coma leading to death. CSF: pressure slightly elevated with presence of lymphocytes and plasma cells, glucose usually low with moderately high protein levels. CT scans were non specific. Clinical course insidious and may mimic bacterial meningitis or tuberculous meningitis. Post mortem pathological examinations showed that acanthamoeba produced a chronic or subacute granulomatous meningioencephalitis. Acanthamoebic trophoziotes and cysts were found within the hemorrhagic necrotic cerebral tissues. In some cases there is severe angitis and fibrinoid necrosis of the vascular wall with perivascular cuffing by lymphocytes and plasma cells. Trophozoites and cysts were found within the perivascular space.Acanthamoeba is an opportunistic pathogenic free living amoeba which can produce fatal GAE.