PAM is an acute infective disease caused by Naegleria fowleri and characterized by a necrotic, hemorrhaging inflammation of the brain and leptomeninges. N. fowleri is an ameba that lives in natural freshwater and infects humans via the inhalation of contaminated water during swimming or by rinsing nasal passages with unboiled or unfiltered tap water. To this date, there have been more than four hundred reported cases of N. fowleri worldwide, of which there are only a dozen survivors. PAM targets mostly children and young adults during hot summer months. N. fowleri invades the brain tissue via the olfactory nerves and bulbs, where it causes inflammation with predominance of neutrophils, macrophages and few eosinophils. Clinical symptoms are unrecognizable from symptoms of bacterial or viral meningoencephalitis, such as headache, high fever, nuchal rigidity, vomiting and nausea. Agitation, photophobia, diplopy, lethargy and confusion occur in later stages, while the rise of ICP leads to convulsions, brain herniation, coma and, finally, death. The diagnosis is based on the detection of motile trophozoites in the sample of wet-mount CSF under a light microscope and the confirmation of the diagnosis via PCR and the detection of N. fowleri DNA. Treatment is based on multimodal antimicrobial therapy that includes amphotericin B which shows synergistic activity with azithromycin and miconazole. Clotrimazole, itraconazole, fluconazole and ketoconazole, chloramphenicol and miltefosine can also be included in the therapy. Elevated ICP should be aggressively treated by medication and, if needed, by surgical intervention. Prevention of PAM requires adequate pool chlorination, monitoring of freshwater inhabited by N. fowleri and caution of every individual while swimming in freshwater or rinsing nasal passages in therapeutic or religious purposes.