“… 2 , 3) While leptomeningeal dissemination can be seen as a late manifestation of systemic lymphoma or synchronously with primary central nerve system malignant lymphoma (PCNSL), lymphoma isolated to the leptomeninges, which is categorized as primary leptomeningeal malignant lymphoma (PLML), is exceedingly rare. 4 , 5) The clinical presentation of PLML varies depending mainly on the locations involved by lymphomatous lesions, and hydrocephalus can also be the trigger for disclosing underlying intracranial malignant lymphoma. 4 , 6 – 10) As for diagnostic workup for PLML, recognizing suggestive findings on magnetic resonance imaging (MRI), such as abnormal enhancement on the surface of brain or ventricular wall, is the key, and definitive diagnosis requires histopathological confirmation via cerebrospinal fluid (CSF) or biopsy.…”