Acute neurological manifestations in patients with Behcet's syndrome are rare yet may lead to devastating outcomes. Distinguishing primary neurological deficits from spontaneous hemorrhagic insults is of particular importance for the prognosis of patients with Behcet's syndrome. Here, we investigate the clinical characteristics, management, and outcomes of nontraumatic hemorrhagic injury in patients with Bechet's syndrome. Following the case presentation, a systematic review of the literature identified cases of spontaneous hemorrhage among patients with Behcet's syndrome. Variables of interest were collected from each article to characterize patient demographics, clinical manifestations, management, and reported outcomes. Additionally, a rare case of nontraumatic intramedullary spinal bleeding in a young male with Behcet's syndrome is presented. Including our case, we analyzed 12 cases of spontaneous bleeding associated with Behcet's syndrome in 12 articles. Patient age ranged from 16 to 71 (median = 36), with a male predominance (n = 11, 91.7%). Involvement of cardiothoracic structures (n = 3, 25%), pulmonary (n = 4, 33.3%), and gastrointestinal or genitourinary vasculature (n = 3, 25%) was most common, followed by extracranial (n = 2, 16.7%) and central nervous system vasculature (n = 1, 8.3%). Clinical presentation varied depending on which specific systems or anatomical structures were involved. Anticoagulation or antiplatelet therapy was mentioned in three cases (27.3%). Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) were noted to be elevated in six cases (54.5%). Most cases were managed surgically (n = 8, 66.7%); four cases were managed conservatively (33.3%). In our case, the patient's intramedullary bleed was allowed to dissolve without further manipulation. Of the reported outcomes, major recovery was achieved in 10 patients (83.3%), and two patients died from aneurysm or pseudoaneurysm rupture (16.7%). New-onset neurological findings in patients with Behcet's syndrome should raise suspicion for possible spontaneous hemorrhage. Our case presents the first reported instance of an abrupt onset of neurological injury secondary to intramedullary spinal cord bleed in Behcet's syndrome. A systematic review of the literature demonstrates no difference in mortality for patients managed conservatively compared to those who undergo surgical treatment.