Reports of spontaneous hemothorax in patients with neurofibromatosis type 1 are scarce despite the severe complication. We herein present the first case of hemothorax in a neurofibromatosis type 1 patient during pregnancy and discuss the difficulty associated with its diagnosis and treatment.
A 39-year-old female at 34 weeks gestation presented with sudden left back pain and dyspnea. Chest radiography revealed massive left pleural effusion. Computed tomography showed bleeding from the intercostal artery. Although the patient appeared hemodynamically stable, the fetus was in a critical condition. Emergency caesarean section was performed within 1 hour. Subsequently, we performed endovascular coil embolization of the intercostal artery. While this intensive treatment saved the patient, her fetus could not be rescued.
Patients with neurofibromatosis type 1 may develop massive hemothorax without gross lesions. In late pregnancy, sufficient infusion and quick hemostasis are essential and can be lifesaving.

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