To present a case report of a child who developed sepsis by Salmonella enteritidis associated with the diagnosis of primary immunodeficiency.A twenty-one month old boy presenting fever and skin lesions, bilateral pneumonia with pleural effusion and septic shock. Salmonella enteritidis was isolated in blood cultures and pleural fluid. The identification of the bacteria suggested the presence of the MIM syndrome. The diagnosis of IL-12Rbeta1 was confirmed after IL-12 and IFN-gamma levels were measured using patient cells in a culture medium. The results showed absence of IL-12 and the IFN-gamma post stimulation using BCG.A severe infection by Salmonella enteritidis is strongly suggestive of an immune system dysfunction. Laboratory tests for humoral, cellular and innate immunity were performed. Interleukin 12 receptor beta1 (IL-12 Rbeta1) deficiency was confirmed after specific laboratory evaluation. The use of INF-gamma is recommended in severe cases.