Acute upper airway obstruction is a potentially life-threatening event. Hemophilia-A is a coagulopathy with high risk for spontaneous bleeding. Here we describe for the first time a spontaneous epiglottic internal hemorrhage leading to upper airway narrowing in a hemophilia-A patient. The patient was admitted to the intensive care unit for airway observation and treatment with factor VIII supplementation. In the first 48 hours there was rapid respiratory improvement. The epiglottic swelling resolved on the 5th day. In this patient no oral intubation or surgical management was needed.