Background: Severe symptomatic factor VII deficiency is very rare. Spleen rupture in a neonate with factor VII deficiency is even rarer. Only one case of hemoperitoneum and intracranial hemorrhage in a neonate with this disease is reported in the literature. Furthermore, the manifestation of hemoperitoneum by abdominal distention, abdominal tenderness, and anemia is often delayed after splenic rupture, and is seldom recognized before the onset of hypovolemic shock.