Introduction: The 2016 revision of the World Health Organization classification of lymphoid neoplasms defined marginal zone lymphoma (MZL) as non-Hodgkin lymphoma (NHL) that includes three distinct subtypes: extranodal MZL of mucosa-associated lymphoid tissue (MALT), nodal and splenic MZL. The MZL is a group of indolent non-Hodgkin B-cell lymphoma (NHL) which accounts for approximately eight percent of all NHL cases and is the third most common histological subtype of NHL after diffuse large B-cell lymphoma (DLBCL) and follicular lymphoma. An important but rare extranodal presentation is the primary central nervous system lymphoma (PCNSL) and it can involve the brain, leptomeninges, intraocular structures and spinal cord in the absence of systemic disease. A small number of dural MZL's cases has been reported and most of them were confined to the meninges with no systemic spread at the time of diagnosis. The PCNSL is very rare and often misdiagnosed as meningioma, because of its location and radiologic appearance. It shows as extra-axial lesions that appear iso- or hypointense on T1 and T2-weighted magnetic resonance imaging (MRI) with intense enhancement with gadolinium administration. The dural tail sign, which is a frequent finding in meningiomas, is also observed in dural MZL. We reported a rare case of a noticeably young female patient with PCNSL arising in the dura mimicking meningioma. Case report: Over 18 months, the 36-year-old female patient was followed by neurosurgery due to the expansive lesion at epidural space. The first signs and symptoms were hemiparesis, hypoesthesia in the left arm, and dysarthria. Investigations were performed, and MRI of the brain revealed a well-defined right extra-axial lesion, measuring 30 x 54 x 56 mm. Initially, no biopsy was indicated and the case was conducted as meningioma. No comorbidity or immune deficiency was found. The patient presented worsening of symptoms, a progression of neurological deficit, severe headache and visual field impairment. Given the size of the lesion, as well as brain compression and worsening of clinical status without a definitive diagnosis, a surgery with resection of the extra-axial lesion was proposed. The patient underwent a frontoparietal craniotomy and the histopathological findings were consistent with the diagnosis of dural extranodal MZL with amyloid deposition. The patient underwent staging for lymphoma and no hepatosplenomegaly or lymph node enlargement was found. Bone marrow biopsy and aspirate were negatives. Discussion: The central nervous system (CNS) involvement is exceedingly rare and sometimes the lesion may resemble other associated CNS disorders such as meningioma. Although initially conducted as a meningioma in a young patient, the hypothesis of CNS lymphoma should be considered as a differential diagnosis, particularly, as in this case, when a more aggressive pattern of the lesion, with bone destruction, is observed. Histological examination is critical for an accurate diagnosis. In this case, the biopsy was fundamental for an appropriate therapeutic approach.