We report the MRI features in a series of four patients with primary angiitis of the central nervous system (PACNS). Based on MRI features, clinical presentation, paraclinical investigations and laboratory tests, including cerebrospinal fluid (CSF) analysis, various differentials were considered. In two patients with MRI findings of cortical, subcortical and deep white matter lesions, lack of mass effect, focal areas of bleed and heterogeneous parenchymal, leptomeningeal or pial enhancement and a normal magnetic resonance angiography, a differential diagnosis of primary angiitis of the CNS was also considered. In all patients, an open brain biopsy was advised to establish a definitive diagnosis of PACNS. Here, we briefly discuss the MRI features, correlation with clinical presentation and paraclinical parameters for the diagnosis of this entity. We also briefly review the literature.