Agnogenic myeloid metaplasia (AMM) is a myeloproliferative disorder characterized by leukoerythroblastosis, tear-drop erythrocytes, extramedullary hematopoiesis with hepatosplenomegaly, and varying degrees of myelofibrosis. The mean age at presentation is about 60 years, and pediatric cases are rare. We experienced a case of AMM in a 9 months old female who was presented with pallor, huge splenomegaly and intermittent fever. Peripheral blood showed leukoerythroblastosis poikilocytosis, and tear drop cells. Bone marrow was difficult to aspirate, and biopsy specimen showed increased reticulin with decreased cellularity, which was compatible with myelofibrosis. We presented a case of AMM with brief review of the literatures.