RESUMO
BACKGROUND: Meningioangiomatosis (MA) is a rare entity characterized by a focal lesion that affects the leptomeninges and the cerebral cortex. CASE DESCRIPTION: We describe a case of a 32-year-old man diagnosed with MA not associated with hamartomatous lesions or with type 2 neurofibromatosis. Magnetic resonance images (MRI) showed an extensive parieto-occipital lesion and another right frontal lesion, initially suggestive of encephalitis. A biopsy of the meninges and brain was performed via a right parieto-occipital craniotomy. The histopathologic diagnosis, complemented by immunohistochemical studies, was MA. CONCLUSION: Diagnosis of MA is very difficult based only on images, therefore lesions compromising the brain cortex, associated or not with calcifications, should be further examined through biopsy so as to have a precise diagnosis.