The identification of the first mutation in the fibrinogen gamma chain gene [12] and the demonstration that the hereditary hypofibrinogenemia, in analogy with AATD, was due to the intrahepatic retention of the mutant protein [13,14,15], have led to the discovery of a new disease, hereditary hypofibrinogenemia with hepatic storage (HHHS), and to the concept of ERSD [1,15,16]. This evidence concerns the gene FGG and Hypofibrinogenemia.