Status epilepticus (SE) is a rare but potentially life threatening complication that women with epilepsy may experience during pregnancy. Refractory status epilepticus (RSE) is defined as an epileptic status which is unresponsive to early standard antiepileptic medical treatments; it has been reported that in pregnant women affected by other diseases (systemic lupus erythematosus, porphyria, vitamin B6 deficiency and cavernous angioma) this status spontaneously resolved only after delivery. We report the case of a primigravida 29-year-old epileptic woman who was admitted to the Emergency Room at 25 weeks of gestation with RSE and a confused mental state. Her Electroencephalography documented repeated seizures not controlled by medical treatments, for this reason she was then sent to the Intensive Care Unit. The patient had no known risk factors. Her cranial magnetic resonance revealed a clivus chordoma, a rare, slow-growing neoplasm, characterized by locally aggressive growth patterns and high local recurrence rates. Spontaneous premature delivery at 26 weeks and three days brought an almost immediate cessation of seizures.