Two new cases of congenital factor VII deficiency are reported. They are two 7-year-old boys belonging to two different kindreds. The main clinical features were: bleeding from the gums, epitaxis (one patient), easy bruising, melena, posttraumatic and spontaneous hemarthrosis. The main coagulation features were: prolonged prothrombin time, normal Stypvencephalin clotting time, normal first stage coagulation tests, normal platelet and vascular tests. The prothrombin time was corrected by the addition of normal serum, factor II or factor V or factor X-deficient plasmas and of the abnormal factor X (factor X Friuli) plasma. On the contrary, the addition of adsorbed normal plasma and of three plasmas with known factor VII deficiency failed to correct the abnormality. The factor VII levels were found to be 2.3 and 2.7% of normal, respectively. The parents of the patients were not consanguineous. But 3 of them, together with other members of the 2 families, were found to have slightly decreased factor VII levels and were considered to be heterozygotes for the defect. These findings are consistent with an incompletely recessive pattern of autosomal inheritance

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