Myeloid leukemias are a heterogenous group of disease characterized by infiltration of blood, bone marrow and other tissues by neoplastic cells of hematopoietic origin. Rarely, patients may present with symptoms from a mass lesion located in soft tissues. The mass lesion represents a tumor of leukemic cells and is called granulocytic sarcoma or chloroma. This is more commonly seen in monocytic subtype with abnormalities of chromosome 11. In general, they are felt to augur a poorer prognosis, with a poorer response to treatment and worse survival. There have been few case reports of orbital and central nervous system (CNS) chloroma in pediatric age group who have done better than only a medullary disease. Here, we present a case of acute myelogenous leukemia M2 who had two relapses one medullary and one extramedullary (CNS relapse with giant CNS chloroma). Patient was reinduced with same drugs and was advised to go for allogenic stem cell transplantation, which he could not. Subsequently presented with features of raised Intracranial tension (ICT) and was diagnosed to have extramedullary relapse in the form of giant CNS chloroma. He was treated with surgical debulking of the tumor, fludarabine, AraC, idarubicin, and G-CSF chemotherapy protocol (FLAG‑IDA) chemo, and local intraspinal triple therapy 5 years back and stands cured at present.