BACKGROUND Von Recklinghausen's disease, also known as Type 1 neurofibromatosis (NF1), is a genetic disorder characterized by skin tumors, neurofibromas of multiple organs and vascular abnormalities. Spontaneous thoracic hemorrhage is a rare but potentially fatal consequence of this disorder. After a review of the literature over the last 10 years and on the basis of a case study, the aim of this study was to report the challenges of management of this pathology. CASE REPORT We report a rare case of a 45-years-old male with a medical history of neurofibromatosis who complained of a 3-day history of progressive dyspnea. At his admission to the Emergency Department, the patient was hemodynamically stable. A chest computed tomography (CT) scan showed a large left hemothorax with mediastinal shift to the right without active bleeding. A chest tube was introduced, and conservative treatment was followed. Another CT scan performed 2 days later revealed a middle lobar pulmonary embolism on the opposite side. A full treatment of anticoagulation was administered, and the patient was released after 8 days of hospitalization. Three weeks later, a new chest CT scan indicated the absence of vascular aneurysm or source for hemothorax. CONCLUSIONS Our systematic literature review found 15 articles which were described as early as 2005. To our knowledge, endovascular treatment produces the best immediate successful result (100%) and may be used in adjunction with video-assisted thorax surgery (VATS) or thoracic drainage to optimize outcomes. In the present case, conservative treatment showed a good result despite anticoagulation for pulmonary embolism. The endovascular approach seems to be the most promising, but treatment needs to be tailored to each individual patient.