A case of recurrent pilocytic astrocytoma with leptomeningeal dissemination (LMD) is described. A cerebellar tumor was diagnosed in a 3-year-old boy, in whom resection was performed. When the boy was 6 years of age, recurrence was treated with surgery and local radiotherapy. At age 13 years, scoliosis was present, but the patient was asymptomatic. Twelve years after initial surgery LMD was demonstrated in the lumbar spinal region without recurrence of the original tumor. This tumor also was subtotally removed. During the procedure, a hematoma was observed adjacent to the tumor, but the border was clear. Histological examination of the spinal cord tumor showed features similar to those of the original tumor. There were no tumor cells in the hematoma. The MIB-1 labeling index indicated no malignant change compared with the previous samples. Radiotherapy was performed after the surgery. The importance of early diagnosis and management of scoliosis is emphasized, and the peculiar pattern of dissemination of the pilocytic astrocytoma and its treatment are reviewed.