Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic disease of abnormally activated complement. FLAER diagnosis is a higher sensitive and specific method, which makes PNH patients to be early discovered and treated. Non-typical symptoms including thrombosis, pulmonary hypertension and chronic kidney disease in PNH have been caused increasing attention. Eculizumab monoclonal antibody has greatly improved the current treatment status of PNH. PNH can be cured thoroughly by allogeneic hematopoietic stem cell transplantation. In this article, the FLAER diagnosis, clinic symptoms and progress of treatment in patients with PNH are reviewed.