en Case Report and Review of Literature Case Report and Review of Literature <div style="text-align: justify;"><span style="font-size:14px;"><span style="font-family:Tahoma;"><span style="line-height:2;"><span style="unicode-bidi:embed"><strong>Background</strong>: Ataxia-telangiectasia is a multi-organ disease. It is due to a mutation of the Exon No. 5 ataxia telangiectasia mutated gene (c.381delA: p.v128fls). Complications including recurrent infections, progressive cerebellar ataxia, and varying degrees of humoral and cellular immunodeficiency arise.&nbsp;<br> <strong>Case Presentation</strong>: We report a 7-year-old girl patient with A-T who developed acute rheumatic fever.&nbsp;<br> <strong>Conclusion</strong>: Rheumatoid disorders and or infectious diseases such as acute rheumatic fever could be observed in A-T patient. </span></span></span></span></div> Ataxia-telangiectasia, Mutation, Immunocompromised patient, Acute rheumatic fever 267 272 http://jpr.mazums.ac.ir/browse.php?a_code=A-10-961-1&slc_lang=en&sid=1 Mohammad Reza Khosravi mohammadrezakhosraviii96@gmail.com 100319475328460010181 100319475328460010181 No Student Research Committee, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran. Ghazal Abbasi Ghazal.abbasi.sh@gmail.com 100319475328460010182 100319475328460010182 No Student Research Committee, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran. Leila Shahbaznejad Leilashahbaznezhad@gmail.com 100319475328460010183 100319475328460010183 No Pediatric Infectious Diseases Research Center, Communicable Diseases Institute, Mazandaran University of Medical Sciences, Sari, Iran. Javad Ghaffari javadneg@yahoo.com 100319475328460010184 100319475328460010184 No Pediatric Infectious Diseases Research Center, Communicable Diseases Institute, Mazandaran University of Medical Sciences, Sari, Iran. Abbas Dabghzade siamakdabbaghzade@yahoo.com 100319475328460010185 100319475328460010185 Yes Pediatric Infectious Diseases Research Center, Communicable Diseases Institute, Mazandaran University of Medical Sciences, Sari, Iran.