Job's syndrome is characterized by the clinical features of fair skin, red hair, recurrent cold staphylococcal skin abscesses with concurrent other bacterial infections and skin lesions. This case report chronicles a classic presentation of Job's syndrome. A brief review of the otolaryngological presentations of immune deficiencies is presented. Other than its relationship to the manifestations of other immunological defects, Job's syndrome is of interest to the otolaryngologist because of the head and neck infections represented in its clinical expression. Familiarity with phenotypic and laboratory manifestations of such diseases will enable earlier recognition and treatment of this syndrome.