Neurofibromatosis type 1 (NF-1) is an autosomal dominant disease characterized by multiple dermatological disorders. The less frequent manifestations are vascular abnormalities. Spontaneous rupture of these pathological vessels is a rare but potentially life-threatening complication; this necessitates immediate and decisive intervention. Unstable patients should be managed with aggressive surgical intervention as soon as possible to avoid mortality. We encountered a case of massive hemothorax in a 62-year-old female with neurofibromatosis type 1 as a result of spontaneous rupture of right intercostal artery, which leads to lethal outcome despite extensive surgical intervention as well as intensive care measures.