Chédiak-Higashi syndrome is characterized by varying degrees of oculocutaneous albinism, recurrent infections, bleeding disorders and variable neurological involvement. It is a rare autosomal recessive disorder. The treatment consists of bone marrow transplantation, which corrects the immunologic and hematologic defects. Nevertheless, oculocutaneous and neurological changes persist. Untreated patients die as the result of bacterial infections or develop »accelerated phase« lymphoproliferation.