Intracranial hemorrhage (ICH) is an uncommon complication of hemophilia in the 1st year of life and most often is reported after head trauma or birth trauma. Spontaneous ICH unrelated to birth or head trauma is rare at any age, especially in the 1st year of life. We describe a 3-month-old infant who presented to the emergency department (ED) with a spontaneous ICH as the presenting sign of hemophilia B. We review the literature and discuss the ED evaluation and management of hemophiliacs with ICH.