Hemolytic anemia is a common clinical problem with diverse etiology. 10-year-old boy presented with severe pallor. He had hemolytic facies, hepatosplenomegaly & deformity of small joints. Investigations revealed DAT-ve anemia, reticulocytosis and low haptoglobin. BM showed erythroid hyperplasia. Past history revealed recurrent fever, rash, hepatosplenomegaly, lymphadenopathy and poly arthritis diagnosed as systemic-JIA and MAS necessitating admission twice. Hence, a diagnosis of hemolytic anemia with auto inflammatory disease was considered. Whole Exome revealed compound heterozygous mutation in CECR1 gene. DADA2 is a syndrome with varied manifestations including vasculitis, hematological and immunological abnormalities. Clue in our patient was presence of periodic fever.