Spontaneous spinal epidural hematoma (SSEH) is a rare disorder that can present as an acute onset of pain and radicular symptoms that mimic disk herniation. Primary spontaneous pneumothorax (PSP) can be defined as presence of air in the pleural space without apparent underlying lung disease or trauma. We describe a rare case of SSEH in cervical spine (C5-7) presenting with a novel association, a bilateral PSP, which has never been documented before. Both SSEH and spontaneous pneumothorax, though rare, can be associated with vasculopathy and our patient had signs of vasculopathy of intracranial vessels and pulmonary vasculature. Vasculopathy also needs to be considered while evaluating a case of PSP without emphysema-like changes (ELCs) and if diagnosed as the cause, pleurodesis could be considered as a part of the management to avoid the sequel of recurrent pneumothorax and prolonged ICU stay in this subset of patients. Thakkar K, Ajayan N, . A Mysterious Case of Spontaneous Cervical Epidural Hematoma and Bilateral Primary Spontaneous Pneumothorax caused by a Rare Etiology. Indian J Crit Care Med 2019;23(4):186-187.

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