Aplastic or twiglike middle cerebral artery (Ap/T-MCA) is a rare cerebrovascular anomaly correlated to stroke. The most common characteristic of the disease is intracerebral hemorrhage. However, treatment measures that can prevent rebleeding have not yet been established. We sought to validate whether extracranial-intracranial (EC-IC) bypass is effective in preventing recurrent intracerebral hemorrhage in patients with Ap/T-MCA. Ap/T-MCA was defined on the basis of the following criteria: 1) M1 occlusion with plexiform vessels, 2) unilateral MCA involvement without progression to the contralateral side, 3) no significant stenosis in the M2 segment, and 4) no stenoocclusive lesions in other intracranial major arteries. The clinical features, surgical procedures, radiologic changes, and treatment outcomes of patients with Ap/T-MCA who presented with ipsilateral intracerebral hemorrhage at our institution from April 2004 to December 2019 were retrospectively reviewed. Seven patients fulfilled the criteria for hemorrhagic Ap/T-MCA. Female preponderance was observed, and the mean age at the time of onset was 38 (range: 28-47) years. Three patients presented with pure intraventricular hemorrhage and 4 with intraparenchymal hemorrhage. Of the 4 patients, 2 had intraventricular extension. All patients underwent EC-IC bypass and did not present with stroke during the follow-up period (median: 36 [range: 5-140] months). Follow-up magnetic resonance imaging revealed regression of the twiglike vessels or periventricular choroidal anastomosis within 1 year after surgery in all patients. EC-IC bypass may be a treatment option that can prevent rebleeding in patients with hemorrhagic Ap/T-MCA.