Introduction: Iron deficiency anemia (IDA) is highly prevalent among Sri Lankan children, particularly in the 6–12 month and 1–2 year age groups, and typically responds well to oral iron therapy . Iron-refractory iron deficiency anemia (IRIDA) is a rare autosomal recessive disorder caused by mutations in the TMPRSS6 gene, resulting in inappropriately elevated hepcidin levels and impaired intestinal iron absorption. Case presentation: A two-year-old girl presented with lethargy and easy fatigability . There wasno history suggestive of chronic illness, malabsorption, or blood loss. Examination revealed marked pallor with normal growth parameters and no hepatosplenomegaly . Investigations demonstrated severe microcytic hypochromic anemia (hemoglobin 2.2 g/dL) with low serum iron, low transferrin saturation, and low serum ferritin. Peripheral blood smear findings wereconsistent with severe iron deficiency anemia. Stool occult blood testing and inflammatory markers were normal. Alpha-thalassemia gene analysis and Meckel’s scan were negative. She received blood transfusion initially followed by adequate oral iron therapy , but showed no significant hematologic improvement. However, a marked rise in hemoglobin was observed after initiation of intravenous iron. She remains clinically well with normalized hemoglobin levels and normal growth while maintained on regular intravenous iron therapy . Conclusions: IRIDA should be considered in children with persistent microcytic anemia unresponsive to appropriate oral iron therapy . Early recognition enables timely institution of intravenous iron treatment and facilitates genetic counseling.