Life-threatening splenic rupture is rare in neonates with severe hemophilia. There are only 3 cases of splenic rupture in neonates with hemophilia reported in the literature. We present the case of an infant, born to a hemophilia A carrier mother. The infant was asymptomatic until discharge at 48 hours of age, but presented on the third day of life with shock, abdominal distension, and severe anemia. Computed tomography of the abdomen confirmed the diagnosis of splenic rupture with hemoperitoneum. The infant recovered after extensive supportive care surgery and factor replacement.