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Abstract
Optic glioma is a rare benign tumour predominantly occurring in childhood, frequently associated with Neurofibromatosis Type 1 (NF1). It threatens vision and—if extending suprachiasmatically—the life of the patient. Symptoms are visual loss, orbital signs including motility disorders and/or nystagmus in suprachiasmatic lesions. There is no therapy approved in studies with highest evidence. Radiotherapy is most effective in stopping tumour progression but has serious side effects, such as brain developmental anomalies, moya moya syndrome and secondary tumours. Therefore in children younger than 9 and patients with NF1, chemotherapy with vincristine and carboplatine is preferred. Surgery plays only a role in the debulking of large tumours, cosmetic correction or definitive removal if visual function is lost. Therapeutic decisions should be based on reliable function testing.