Kafle, RishikeshBhatta, AnweshGami, SumitSapkota, AbhinSharma, DipeshYadav, ArabindraChikanbanjar, Vijaya Kumar2026-02-132026-02-132021https://hdl.handle.net/20.500.14572/4735Rishikesh Kafle Department of Pediatrics, Kathmandu Medical College Teaching Hospital, Sinamangal, Kathmandu, Nepal Anwesh Bhatta Department of Pediatrics, Kathmandu Medical College Teaching Hospital, Sinamangal, Kathmandu, Nepal https://orcid.org/0000-0002-6100-545X Sumit Gami Patan Academy of Health Sciences, Lalitpur, Nepal. https://orcid.org/0000-0002-1449-7105 Abhin Sapkota Vayodha Hospitals Private Limited, Balkhu, Kathmandu, Nepal https://orcid.org/0000-0002-4146-7016 Dipesh Sharma Karuna Hospital, Budanilkantha, Kathmandu, Nepal https://orcid.org/0000-0002-9032-2219 Arabindra Yadav Department of Pediatrics, Kathmandu Medical College Teaching Hospital, Sinamangal, Kathmandu, Nepal https://orcid.org/0000-0003-4301-7200 Vijaya Kumar Chikanbanjar Department of Pediatrics, Kathmandu Medical College Teaching Hospital, Sinamangal, Kathmandu, Nepal https://orcid.org/0000-0001-9062-0083Abstract: Macrophage activation syndrome is a rare but a life threatening condition commonly associated with Systemic Juvenile Idiopathic Arthritis. Its clinical presentation includes fever, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia and impaired liver enzymes. The symptoms are alarming yet non-specific and often lead to a delayed diagnosis. A 12 year male presented with a history of intermittent fever and was started on antibiotics but failed to respond after several days of hospital stay. After a series of investigations to rule out multiple diagnoses he was diagnosed as a case of Macrophage Activation Syndrome secondary to Systemic onset Juvenile Arthritis and was treated with steroids.en-USuvenile arthritismacrophage activation syndromesteroids.Article