Hyperimmunoglobulinemia E (Job or Buckley) syndrome is an immunodeficiency disease associated with recurrent pyogenic infections, chronic eczematoid dermatitis, and mucocutaneous candidiasis. Association of this condition with osteoporosis and recurrent fractures is presented by a case report and by review of other reports describing similar findings. Other immunodeficiency syndromes with associated skeletal abnormalities are discussed.