Object. The goal of this study was to identify the origins of spontaneous fusiform middle cerebral artery (MCA) aneurysms. Methods. One hundred two cases of spontaneous fusiform MCA aneurysms were reviewed, including 40 from the authors' institutions and 62 identified from the literature. The mean age at symptom onset was 38 years, and the male/female ratio was 1.4:1. At presentation, the MCA lumen was stenosed or occluded in 12 patients, focally dilated in 57, and appeared "serpentine" in 33. Most lesions originated from the M, or M, segments, and most (80%) presented with nonhemorrhagic symptoms or were discovered incidentally. The presenting clinical features correlated with morphological findings in the aneurysms, which could be observed to progress from a small focal dilation or vessel narrowing to a serpentine channel. Hemorrhage was the most common presentation in small lesions; the incidence of bleeding progressively diminished with larger lesions. Patients with stenoses or occluded vessels most often presented with ischemic symptoms, and occasionally with hemorrhage. Giant focal dilations or serpentine aneurysms were rarely associated with acute bleeding; clinical presentation was most often prompted by mass effect or thromboembolic stroke. Conclusions. Analysis of results after various treatments indicates that for symptomatic lesions, therapies that reverse intraaneurysmal blood flow and augment distal cerebral perfusion are associated with better outcomes than other strategies, including conservative management. Based on the spectrum of clinical, pathological, neuromiaging, and intraoperative findings, dissection is proposed as the underlying cause of these lesions.