Chronic myelogenous leukemia (CML) is the most common of all leukemia constituting 15–20% of all leukemia. The clinical course of the diseases runs in two to three phases, initial chronic phase followed by accelerated phase or blast phase. Blast phase most commonly presents clinically as fever, splenomegaly, and bone pain. Here, we present a case of CML in blast phase presenting with central nervous system manifestation in a 55-year-old patient with a brief review of the literature.