This study reports on the long-term course of 11 patients (6 girls, 5 boys) with childhood onset schizophrenia (COS, age at onset < 10 years). Patients were examined twice (mean follow-up period 38 years after onset). The premorbid development is assessed in terms of the Modified Premorbid Adjustment Scale (M-PAS) and additionally described by distinct psychopathological categories. The psychopathology at the onset of psychosis and at the second follow-up examination was assessed by categorical application of the Positive and Negative Syndrome Scale (PANSS). The outcome was rated with the Disability Assessment Score (DAS). The course of psychotic episodes and intervals between them is presented according to DSM-IV subtype classifications. Ten of 11 patients presented premorbid developmental peculiarities that were not adequately covered by the M-PAS subscales. Whereas in the 4 patients with acute onset of psychosis the positive PANSS-type was predominant, in the 7 patients with an insidious onset the negative PANSS-type prevailed. The nature of the diagnostic subtypes varied markedly across the course of the illness. In case of a continuous predominant catatonic symptomatology the outcome was poor. Detailed case descriptions help to illuminate the heterogeneous psychopathology of COS. Various temporary premorbid behavioral peculiarities were precursors of COS. A differentiation between premorbid and prodromal signs proved to be arbitrary. Our results contradict the assumption that COS is characterized only by a negative symptomatology.