We report a case of von Recklinghausen's disease associated with multiple intracranial arterial occlusion ("moyamoya-like") and a fusiform aneurysm. A 28 years-old man with type I neurofibromatosis presented with syncope, complex partial seizures and mental deterioration. After an acute headache episode associated with meningeal signs, drowsiness, and hemorrhagic CSF, the patient was evaluated with cranial CT scan, MRI and angiogram that revealed an obstructive arteriopathy compatible with moyamoya disease and a fusiform aneurysm in the posterior circulation. The authors discuss the clinical and radiological findings and the therapeutic decision in this case comparing with the few similar reports in the medical literature. Recognition of an underlying rare genetic disorder may be of considerable importance in young patients presenting with seizures.