Paroxysmal nocturnal hemoglouinuria(PNH) is a disorder of the hematopoietic stem cell that results in the production of a clone of defective red blood cells in circulating lines. Clinical and laboratory observations were made in a case of PNH admitted to Chonnam University Hospital. The patient was 16-year-old female who complained general weakness and headache. The peripheral blood revealed anemia with reticulocytosis. Whole blood screening test, sucrose hemolysis test and Ham's test were positive. Serum bilirubin was moderately increased. On urinalysis, marked hemoglotinuria and hemosiderinuria were shown. The bone marrow aspiration revealed decreased cellularity with marked erythroid hyperplasia. She was treated with transfusion of packed red cells and discharged with partial alleviation of clinical manifestation.