Thrombosed arteriovenous malformations (AVMs) are the predominant type of occult vascular malformation and do not seem to differ significantly in clinical or radiographic presentation from other types of occult vascular malformations. Thrombosed AVMs and occult vascular malformations occasionally present with symptoms secondary to subacute or occult hemorrhage that requires operation. The histopathology of thrombosed AVMs and occult vascular malformations does not seem to have prognostic significance. Five patients with histologically verified thrombosed AVMs are reported. Two patients had previous incomplete resection of histologically proven thrombosed AVMs and presented with recurrence and bleeding. Two patients presented with seizures and headaches, and one patient presented with hemiparesis and headache. All histology specimens had evidence of hemorrhage. The pathological findings of these lesions seem variable; two specimens contained a mixture of cavernous angioma and AVM. Two previously resected lesions had been defined histologically as thrombosed AVMs. Successful excision of the lesions was accomplished in four patients, and one patient had stereotactic biopsy. The magnetic resonance imaging characteristics of lesions seem to add a degree of specificity over computed tomography and angiography. We have found that the literature poorly describes the natural history and histology of these lesions. Conservative management for seizures and headaches due to thrombosed AVMs may not be warranted because of a propensity for hemorrhage and recurrence.