Our aim was to report our experiences of pediatric macrophage activation syndrome (MAS) patients treated with anakinra and to review previous studies reporting anakinra treatment in pediatric MAS patients associated with systemic juvenile idiopathic arthritis (sJIA) or autoinflammatory diseases (AIDs). The study group consisted of pediatric MAS patients due to sJIA or AIDs, followed up in the Pediatric Rheumatology Unit of Hacettepe University between January 2015 and January 2017 and treated with anakinra (anti-IL1). We conducted a systematic review of the published literature involving pediatric MAS patients associated with sJIA or AIDs, treated with anakinra. Thirteen sJIA patients and two AIDs patients were included the study. Nineteen MAS episodes were observed in 15 patients. Anakinra (2mg/kg/day) was started in with a median 1day after admission. Clinical symptoms resolved, and laboratory findings normalized within median (minimum-maximum) 2 (1-4) and 6 (4-9) days, respectively after the introduction of anakinra. Steroid treatment was stopped in a median of 10 (4-13) weeks after the initiation of anakinra treatment. Patients were followed up for a median of 13 (6-24) months. Two patients developed recurrent MAS episodes when the anakinra dose was reduced, while the other patients achieved remission. In the literature review, we identified nine articles, describing 35 pediatric MAS patients associated with sJIA or AIDs and treated with anakinra. Except for two, all the patients reached remission. Our study and systematic literature review may help to improve the knowledge on the role of anakinra treatment in the management of MAS.