Abstract Background Isolated spinal artery aneurysms are extremely rare, and their pathogenesis, clinical presentation, and treatment strategies are poorly established. We report only the second case of a patient with an isolated posterior spinal aneurysm and concurrent left thalamic infarct and review the literature to help clarify treatment strategies of isolated spinal aneurysms. Case presentation A 49-year-old patient presented with acute onset walking difficulty followed by diaphoresis, back and abdominal pain, and paraplegia. Imaging was notable for a hemorrhagic spinal lesion with compression at T12 through L4 and an acute left thalamic infarct. Surgical exploration revealed an isolated posterior spinal artery aneurysm. The aneurysm was surgically resected and the patient had partial recovery six months post-operatively. Conclusions Isolated posterior spinal artery aneurysms of the thoracolumbar region are rare lesions that commonly present with abdominal pain, radiating back pain, and lower extremity weakness. Imaging may not provide a definitive diagnosis. The three primary treatment strategies are conservative management, endovascular treatment, or surgical resection. In patients with symptomatic cord compression, immediate surgical intervention is indicated to preserve neurologic function. In all other cases, the artery size, distal flow, morphology, and location may guide management.