Hallgren syndrome is a hereditary disease with autosomal recessive inheritance. Its exact genetic background has not been elucidated so far. From the clinical aspect is comprises association of retinitis pigmentosa, atrophy of the optic nerve, nystagmus and congenital hearing damage combined with neurological and psychiatric symptoms. The authors describe two siblings with the clinical picture of this syndrome. It is a finding not published so far in the Czech literature.

        Key words: :Hallgren syndrome, retinitis pigmentosa, congenital deafness