Although severe combined immunodeficiency is rarely seen seen it can be lethal. Often the symptoms of the disease are present in the first three months of life. If the disease is not diagnosed and treated properly death of the patient is inevitable in the first two years of life. The patient in this case report is a three-month-old girl' presenting recurrent upper and lower respiratory tract infections since last one month was in severe sepsis. We lost her due to the severe cytomegalovirus and other oppurtunistic infections in her 10th day of hospitalization. We present this case because it is important to remember immunodeficiencies can be the cause of severe and recurrent infections in early years of life.