Evans syndrome is a chronic and recurrent disease often resistant to multiagent therapy. Spontaneous intracranial hemorrhage is a rare but life-threatening complication in children with Evans syndrome. The authors report the fatal course of a child with chronic thrombocytopenia who presented a spontaneous intracranial hemorrhage. The case indicates that this potentially fatal event may occur even in children with a moderate grade of chronic thrombocytopenia. Early recognition and aggressive management is required for an adequate management of this rare complication.