Next to VKCFD1, a second autosomal recessive coagulation factor deficiency exists, VKCFD2 (OMIM#607473), caused by VKORC1 (vitamin K epoxide reductase complex, subunit 1; OMIM*608547) mutations and is also characterized by a deficiency of all VK-dependent clotting factors. The gene discussed is VKORC1; the disease is coagulation protein disease.