To identify predictors of intellectual development in a cohort of children with permanent primary congenital hypothyroidism. Cohort study with intellectual development as the outcome. Thirty-one consecutive newborns with permanent primary congenital hypothyroidism diagnosed by a screening programme were recruited from the outpatient clinic of a district hospital and underwent psychometric evaluation with the Wechsler Intelligence Scales after a minimum follow-up of 4 years. Eight of the 31 patients (25.8%) presented impaired intellectual development (full-scale intellectual quotient < 85), and one of them presented mental deficiency (full-scale intellectual quotient </= 69). The following were associated with worse prognosis: initial serum T4 levels </= 32.18 nmol/l, treatment beginning after 30 days of age, fewer than seven clinic visits during the first year of life, living in rural areas, nonintellectual parental occupation and little parental schooling. In a multiple regression analysis, only maternal schooling (B = 0.401; beta coefficient = 13.053, P = 0.063), number of clinic visits during the first year of life (B = 0.382; beta coefficient = 4.145, P = 0.047) and initial serum T4 (B = 0.287; beta coefficient = 1.336, P = 0.089) remained significantly associated with full-scale intelligence quotient scores. Maternal schooling, number of visits during the first year of life and baseline T4 levels were the main predictors of cognitive outcome in this cohort of patients.