X linked agammaglobulinemia (XLA) is rarely reported from developing countries especially from South East Asia. It appears that × linked agammaglobulinemia is less common in certain ethnic groups. It is very uncommon in black people in USA and South Africa. In multiracial Malaysia we have documented five XLA in Malays and Indians but not in the Chinese that constitute about 31% of the population. First degree relatives afflicted with XLA or other primary immunodeficiencies occurred more often in our study. All showed lung involvement although the etiologic organisms involved were atypical, being Gram negative.