A child with incontinentia pigmenti (Bloch-Sultzberger syndrome) had recurrent pneumococcal meningitis and pneumococcal bacteremia with associated subdural hematomas. Immunologic evaluation revealed defective neutrophil chemotaxis with normal neutrophil chemiluminescense. In addition, lymphocytes showed a depressed proliferative response to phytohemagglutinin stimulation. An immunologic defect may prove to be part of this syndrome.