Venetoclax, a selective inhibitor of BCL2, has shown promising efficacy in chronic lymphocytic leukemia (CLL) both as single agent or in combination therapy. Central nervous system involvement (CNSi) is a rare complication of CLL and is reported in approximately 0.4% of patients. Therapeutic guidelines have not yet been established and prognosis for these patients is poor. Few cases of CLL with CNSi have been reported until now, and ibrutinib was successfully used due to its ability to penetrate the blood-brain barrier.