Congenital factor XIII (FXIII) deficiency is a rare coagulopathy with the highest incidence in Iran. Iranian patients with FXIII deficiency (FXIIID) presented high rate of bleeding episodes, some of them are major cause of disability and mortality among these patients. Hemarthrosis and intracranial hemorrhage (ICH) can affect activity and social productivity of patients. ICH, recurrent miscarriage and umbilical cord bleeding are the major cause of mortality. Hematoma, and prolonged menstrual bleeding as well as post-surgical bleeding are other significant bleeding in Iranian patients with FXIIID. Present of severe life threatening bleeding episodes and other notable bleedings, can significantly reduce working activities and social productivities of patients. Although Iranian patients with FXIIID, experienced significant diseases related complications, early diagnosis accompany by appropriate therapeutic regimes can prevent most of these problems.