Factor XIII deficiency is a rare hemorrhagic disorder that can cause spontaneous intracranial hemorrhage and bleeding after surgery. The diagnosis of factor XIII deficiency is difficult before surgical interventions, because coagulation parameters are normal in these patients. Important clinical findings are postsurgical bleeding and recurrent spontaneous intracranial hematomas. These findings should raise the clinical suspicion of factor XIII deficiency. Therefore, diagnosis of factor XIII deficiency is very important for neurologists and neurosurgeons in terms of reducing mortality and morbidity. We present an 8-month-old female patient who developed subdural hematoma after ventriculoperitoneal shunt surgery but not bleeding after choroid plexus papilloma due to FXIII deficiency.