Lingual hematoma (LH) is an uncommon and potentially life-threatening condition due to its tendency to cause upper airway obstruction. It usually occurs as a result of trauma (motor vehicle accidents, grand mal seizures or traumatic tracheal intubations) and rarely spontaneously in cases of patients with inherited or acquired coagulopathies, high blood pressure, hematological disorders, or vascular malformations. Herein, we report the first case, to our knowledge, of a traumatic massive lingual hematoma in a patient with Systemic lupus erythematosus (SLE) secondary to tongue biting after neurological deterioration, hypertensive crisis and multiple tonic clonic seizures during hemodialysis for chronic renal failure.