Griscelli syndrome is an inherited condition characterized by hypopigmented skin, silver-gray hair, neurological and immune system abnormalities. There are three types with different signs and genetic causes. Patients with type 2 are prone to recurrent infections and they develop hemophagocytic lymphohistiocytosis (HLH). The prevalence is <1 / 1 000 000. The onset of the signs of the disease is in infancy and death happens in childhood if untreated. Diagnosis can be made by clinical presentation, hair shaft study, imunological laboratory finding and genetic tests. Treatment includes bone marrow transplantation.