Abstract: Objective To investigate the clinical features, diagnosis and treatment of inherited coagulation factor Ⅶ deficiency (FⅦD) in newborns. Methods The clinical manifestations, diagnosis and treatment process, and genetic testing of two cases of neonatal FⅦD diagnosed at Guangzhou Women and Children's Medical Center were retrospectively analyzed. The literature about the clinical features of FⅦD in the neonatal period was reviewed and summarized. Results Two female cases of FⅦD presented with severe gastrointestinal tract and intracranial hemorrhage. Their laboratory tests were characterized by repeated and non-VitK1-dependent prothrombin time (PT) prolongation and normal activated partial thromboplastin time (APTT). The factor Ⅶ activity was 1.5% and 3% respectively. These two cases both died of a large area of intracranial hemorrhage at 31 days and 6 months after birth respectively. Gene sequencing results showed a homozygous mutation in the F gene IVS7+1G>T cleavage site in both two cases. In addition, we reviewed 24 cases of full-term neonatal FⅦD of both sexes. Among them, 22 cases were reported by the literature, and 2 by this report. Most of newborns(73.7%) had the first onset of the disease mostly within 7 days after birth. The main initial symptoms of disease onset included gastrointestinal bleeding (blood stool, vomiting blood) (9/24), nervous system signs (drowsiness, convulsions, poor response) (8/24) and pale skin(7/24) .The proportion of intracranial hemorrhage was high(23/24). PT was significantly prolonged, and FⅦ activity was significantly decreased(≤ 5% accounting for 83.3%). The incidence of mortality and disability was 70.8%(17/24). Fourteen of 24 babies got positive F gene diagnosis. Conclusion FⅦD is a rare disease in the neonatal period. Once the disease occurs, life-threatening bleeding is prone to occur and the prognosis is always poor. FⅦD can be clinically confirmed with the presence of prolonged PT that is difficult to correct and significantly decreased FⅦ activity. As mutations in some sites of F gene can cause severe bleeding, a genetic diagnosis contributes to prenatal diagnosis.