The first case was 7-month-old immunodeficiency girl in whom the diagnosis of Acanthamoeba pneumonia was established by culture of a bronchial washing. The patient had been ill for a month when she was admitted due to neonatal thrombocytopenia with respiratory difficulty and treated with gammaglobulin and steroid. Her chest X-ray showed diffuse alveolar consolidation on the left lung with interstitial hazziness and a partial sign of hyperinflation on the right lung. Laboratory tests showed that the Candida antigen was negative and Pneumocystis carinii was not detected. Mycoplasma antigen was negative. All the immunoglobulin levels (IgG, IgA, IgM) were below the normal range. Five days later the patient expired. The second case was an immunosuppressed 7-year-old boy in whom Acanthamoeba trophozoites were found in the skin biopsy, followed by meningitis leading to death. About five days after a laceration on the region of the left eyebrow, a painful bean-sized nodule developed at the suture site and it was treated with antibiotics and corticosteroid. The skin biopsy showed severe inflammatory cell infiltration. Trophozoites were scattered near the blood vessels throughout the inflammatory zone. From one weak prior to admission, the patient had suffered from vomiting, indigestion and mild fever. Skin nodules with tenderness appeared all over his body surface. Examination of cerebrospinal fluid showed clear, Gram stain was negative, bacterial culture negative, India ink preparation negative, and organism on wet smear negative. On admission day 10, focal seizure of the left extremity occurred. Brain CT revealed calcific density on the left parietal lobe area and hypodensity on the left basal ganglia. He became comatous and died immediately after discharge. Until now in Korea, two cases that are described in this paper, one Acanthamoeba meningoencephalitis case and seven Acanthamoeba keratitis cases including two unreported keratitis cases that are reported in this paper have been presented.