INTRODUCTION: Infection of the Central Nervous System by free living amebas is an unusual event, 344 cases have been reported to date. The disease becomes evident in two different clinical fashions: Primary Amebic Meningoencephalitis (PAM) caused by Naegleria fowleri and Granulomatous Amebic Encephalitis (GAE) induced by Spp. of Acanthamoeba and Balamuthia. CLINICAL CASES: The authors report three new cases from Venezuela. Case 1. 34 years old man, with a chief complaint of general malaise, headache and fever, a diagnosis of common cold was made and the patient was treated as such, he did not improve and was admitted to the hospital with deterioration of his clinical status; the patient died 10 days after the onset of his illness which was determined to be GAE produced by Balamuthia mandrillaris. Case 2. 8 years old female admitted to the hospital because of fever, headache and generalized seizures of sudden onset; neurocysticercosis was diagnosed and following improvement the patient was discharged and readmitted on two occasions because of relapse and worsening of her illness, she died 2 months after the onset of her disease that was diagnosed by autopsy as GAE due to Balamuthia mandrillaris. Case 3. 16 years old male, previously healthy, who following immersion in a water tank was admitted to the hospital because of meningeal irritation that progressed to coma and death in a 7 day lapse; autopsy revealed PAM by Naegleria fowleri. The two cases of GAE due to Balamuthia mandrillaris occurred in apparently immunocompetent individuals, contrary to the statement that these microorganisms are opportunistic. CONCLUSION: We believe that neurological infection by amphizoic amebas is being underdiagnosed, probably due to ignorance regarding this pathology or because of a very low autopsy rate in most countries.