Idiopathic pulmonary hemosiderosis is usually thought of as a chronic relapsing disease. Sudden and unexpected death occurred in a 22-year-old woman with this condition. Only one similar case in an adult was found in the literature. Six cases of fulminant idiopathic pulmonary hemosiderosis were also found but were not accepted as sudden and unexpected deaths by the authors. Our case together with the others offers further evidence that the course of the disease may follow no typical pattern.