Agammaglobulinemia or hypogammaglobulinemia is the most common of primary immunodeficiency disorders. patients have recurrent infections in upper and lower respiratory tract, gastrointestinal tract and also affecting other systems. These disorders need to be differentiated from secondary immunodeficiency due to other diseases affecting the immune system.The high birth rate together with the high consanguinity rates favors the occurrence in Egypt and similar developing countries. Objectives: to characterize patient profile for children with agammaglobulinemia or hypogammaglobulinemia, identify presenting symptoms of illness and complications, evaluated of effect of Intravenous immunoglobulin therapy on immunoglobulin levels and frequency of infection, recognize problems with care and obstacles with compliance, identify impact of illness on child and family. Methods: twenty one cases with antibody deficiency disorders frequenting the primary immunodeficiency clinic at Cairo University specialized paediatrics hospital.Patients were subjected to detailed history taking with emphasis on affecting factors as consanguinity, age, sex, types and frequency of infection through clinical examination and indicated investigations. Results: The age of patients ranged between (8 months-14 years), (90.5%) were males, consanguinity was positive in (71.4%), sinopulmonary infection was the most prevalent presentation in cases, Hypogammaglobulinemia was the highest incidence followed by X-linked agammagloblinemia and Hyper IgM consecutively, Correlations were conducted between IgG level and Pulmonary Function Tests with Computerized Tomography findings and revealed no significance, There was a statistically significant difference between IgG pre-and post transfusion levels with a P value < 0.001. Conclusion: Primary immunodeficiency disorders may have very subtle presentations early in life and need a high index of suspicion to be picked up.