Objective: To investigate the causes and treatment of congenital absence of clotting factor VII. Method: The clinical data of one infant with the disease of congenital absence of clotting factor VII in our department were retrospectively analyzed. Result: The infant was transferred to neonatal ICU because of the skin bleeding for no apparent reason. The determination of coagulation factor VII was C 0.5%, revealing the coagulation factor VII deficiency. The coagulation factor VII C of the infant's parents and sister was respectively 28.5%, 31.0% and 25.2%, indicating the relationship with genetic factors. After repeated infusion of fresh frozen plasma and prothrombin complex, the infant recovered and was discharged from hospital. Conclusion: In clinical work, coagulant function abnormality of neonatal repeatedly, we should check the clotting factor as soon as possible in order to avoid missed diagnosis, misdiagnosis and delay treatment.