Congenital/early blindness is reportedly protective against schizophrenia. Using a whole-population cohort of 467,945 children born in Western Australia between 1980 and 2001, we examined prevalence of schizophrenia and psychotic illness in individuals with congenital/early blindness. Overall, 1870 children developed schizophrenia (0.4%) while 9120 developed a psychotic illness (1.9%). None of the 66 children with cortical blindness developed schizophrenia or psychotic illness. Eight of the 613 children with peripheral blindness developed a psychotic illness other than schizophrenia and fewer had developed schizophrenia. Our results support findings from small case studies that congenital/early cortical but not peripheral blindness is protective against schizophrenia.