Systemic chemotherapy was adopted in Kobe in 1996. In this study the authors analyse the clinical manifestations and results of treatment for patients with retinoblastoma since then. This review is of 43 eyes of 34 patients. Those with follow-up to a minimum of one year were included – 25 monocular and nine binocular cases with a median age at first examination of 13 months (22 days to six years and two months). Three patients had a family history of retinoblastoma. The youngest case was one of these three children. The mean observation period was 10.6. months ±53 (16-211). Clinical presentation at first examination included leukocoria (33) and exotropia (one). There was no correlation between age at first examination and classification stage of retinoblastoma. Nineteen eyes were in the initial enucleation group and 24 eyes in the initial preservation plan. Of the latter, 11 eyes continued preservation and 13 discontinued. Of the preservation cases, seven were enucleated because of poor tumour reduction, five showed tumour recurrence, one was enucleated due to secondary glaucoma, one developed secondary cataract and one patient had multi-organ metastases and died. Systemic chemotherapy with vincristine after 2000 had slightly better outcomes than that with ifosfamide before 1999. The study demonstrated that in cases that eventually need enucleation, chemotherapy can significantly delay this with the advantage that the orbit and eyelid can develop normally first and thus reduce the occurrence of facial defects caused by orbit and lid deformities following early enucleation. Eye preservation treatment was performed for a higher proportion of advanced cases among bilateral cases than monocular cases.