Meningioangiomatosis (MA) represents a vascular hamartoma accompanied by meningothelial cell proliferation. It generally becomes symptomatic with difficult to control seizures, though in some patients it may be asymptomatic. We present the case of a 41-year-old male patient with a newly developed central distal monoparesis of the left leg. Cranial magnetic resonance imaging (MRI) and further diagnostic characterization via (18)F-Fluoro-Ethyl-Tyrosine positron emission tomography ((18)F-FET-PET) indicated a low-grade glioma. Histopathological diagnosis revealed a meningioangiomatosis. The clinical, radiological and neuropathological findings of this rare constellation are described and discussed with the actual literature.