Primary central nervous system lymphoma is a rare and aggressive form of extranodal non‐Hodgkin lymphoma, and its occurrence in pharmacologically immunosuppressed patients has increased over the years. Currently, mycophenolate mofetil (MMF) is one of the recommended treatment options for the induction and maintenance of lupus nephritis. The evidence regarding the development of PCNSL in patients with systemic lupus erythematosus (SLE) treated with MMF is limited to a few case reports. We present the case of a 40‐year‐old woman with a history of SLE who developed PCNSL while treated with MMF for lupus nephritis and a review of all published cases with this association.