Abstract: Objective To investigate the clinicopathological characteristics, diagnosis and differential diagnosis of meningioangiomatosis(MA). Methods The clinical manifestations, radiological,histopathological and immunohistochemical features were analyzed retrospectively in 7 MA patients. Related literatures were reviewed. Results Of 7 patients, 2 were males and 5 were females. The age ranged from 4 to 56 years old with a mean age of 17.86 years old. Five patients presented seizure as the initial symptom. Six cases appeared as mass lesion on neuroimaging, but the radiographic findings of MA may show a variety of features. All lesions, usually involved the cerebral cortex, were composed of two main elements, proliferative small blood vessels and perivascular spindle cells. These blood vessels showed variable degrees of hyalinization and calcification. These perivascular spindle cells exhibited some histological features, similar to meningothelial cells. The nuclei were round to elongated, with bland chromatin. The cell membranes appeared fuzzy or indistinct, and eosinophilic cytoplasm. There was no obvious atypia or mitoses. There were reactive gliosis and microcalcification in entrapped cortex. Degenerative changes of neuron were sometimes found, such as neurofibrillary tangles. Immunohistochemically, the proliferative perivascular spindle cells were positive for vimentin in all cases, and were focal positive for EMA and scattered immunoreactive for PR in 3 cases. MIB1 labeling index (MIB1 LI) was low. All patients received surgical treatment without chemotherapy or radiotherapy postoperatively. No recurrence was confirmed during follow-up from 1 to 29 months. All cases were disease-free survival. Conclusion MA is a rare slow-growing intracranial lesion. Histopathological and immunohistochemical features combined with radiological are helpful in diagnosis. MA patients have a good prognosis after surgical treatment.