Background: Severe symptomatic factor VII deficiency is a very rare condition. Spleen rupture in a neonate with factor VII deficiency is even rarer. There is only one case of hemoperitoneum and intracranial hemorrhage in a neonate with factor VII deficiency reported in the literature. In addition, 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. Case: We present the case of a term 24-hour-old newborn who was admitted to the neonatal intensive care unit for pallor and vital distress. He was diagnosed with perinatal splenic rupture and underwent splenectomy owing to hemodynamic instability. A cephalohematoma from a traumatic delivery with serial compressions on the mother’s abdomen during labor, had also been noted upon admission. In addition to these findings, coagulation abnormalities were noticed on serial testing, with consistently prolonged prothrombin time and normal partial thromboplastin time. Subsequent workup eventually led to the diagnosis of congenital factor VII deficiency, with a 2% activity level of the factor in the plasma. Conclusion: Factor VII deficiency is a rare condition with variable presentations (epistaxis, easy bruising, gum bleeding, muscle hematoma, gastro-intestinal bleeding, and central nervous system bleeding). In this condition, there is poor correlation between clinical symptoms and factor VII activity levels in the plasma. However, most severe bleedings usually occur early in infancy. Our term newborn patient presented with two sites of bleeding: the cephalohematoma, and the hemoperitoneum from the ruptured spleen. It is not clear whether these two bleeding incidents were related to his factor VII deficiency; or if they were independent discoveries with no correlation to his coagulation disorder, simply indicating a traumatic delivery.