Tehran University of Medical Sciences Iranian Journal of Allergy, Asthma and Immunology 1735-1502 23 5 2024 10 06 594 599 Zahra Shahraki ghadimi Clinical Immunology Research Center, Zahedan University of Medical Sciences, Zahedan, Iran Simin Sadeghi Bojd Research Institute of Cellular and Molecular Sciences in Infectious Diseases, Ali Ibne Abitaleb Hospital, Zahedan University of Medical Sciences, Zahedan, Iran Nima Parvane Research Center for Immunodeficiencies, Childrens Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Mehdi Atabaki Clinical Immunology Research Center, Zahedan University of Medical Sciences, Zahedan, Iran Ebrahim Alijani Clinical Immunology Research Center, Zahedan University of Medical Sciences, Zahedan, Iran 2024 02 18 2024 07 08 Nephrotic syndrome is characterized by the leakage of protein from the blood into the urine along with the triad of proteinuria, albuminuria, and peripheral edema. Loss of protein leads to the loss of immunoglobulin and complements. X-linked agammaglobulinemia (XLA), or Bruton disease, is a primary immunodeficiency disease caused by a defect in the development of B cells in the bone marrow and a low serum level of immunoglobulins. The present case involves a 12-year-old boy with nephrotic syndrome, osteomyelitis, and recurrent infections. We discovered that he had XLA. This report underscores the importance of considering inborn errors of immunity in cases of protein loss, such as nephrotic syndrome. https://ijaai.tums.ac.ir/index.php/ijaai/article/view/4033 https://ijaai.tums.ac.ir/index.php/ijaai/article/download/4033/2096