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Background and Objectives Primary immunodeficiency (PI) diseases are those occur because of a primary disorder in the immune system. Subjects and Methods This retrospective study was conducted on 70 children with PI diseases referred to the allergy and immunology department of Abuzar Children Hospital in Ahvaz, Iran from 2015 to 2018. Results The most common PI diseases in the majority of male children (56.92%) were severe combined immunodeficiency disease (21.42%) and chronic granulomatous disease (8.57%). Parents in most of patients (83%) were blood relatives. About 66.67% of all death cases (9.23%) was due to severe combined immunodeficiency disease; hypogammaglobulinemia and Griscelli syndrome accounted for about 16.67% of deaths. The most common manifestations were pneumonia, fever, diarrhea, and sepsis. Based on the family history, 33 children had allergies, 9 had malignancy, one had autoimmune disease, and 39 had mothers with a history of abortion. Most of children were under treatment with intravenous immunoglobulin and prophylactic antibiotics, (99.88% and 88.99%, respectively), while 2.81% were under interferon-gamma therapy and transplantation, and 1.40% were receiving corticosteroids and granulocyte colony-stimulating factor therapy. Conclusion The severe combined immunodeficiency disease, chronic granulomatous disease, and Bruton disease are the most common PI diseases in children living in Ahv [ABSTRACT FROM AUTHOR]