Abstract
We reported two cases of the central nervous system (CNS) multiple myeloma (MM) with unusual presentation of sixth nerve palsy. The first patient developed in the setting of newly diagnosed MM and the second patient in the relapsed refractory setting. One underwent surgery, and the other received radiation. Both patients received systemic chemotherapy and noted improvement. We also performed a comprehensive literature review of previously published cases of sixth nerve palsy from MM. This review highlights the importance of recognizing this presentation of CNS multiple myeloma to avoid delays in diagnosis and to get appropriate management in time.