PURPOSE: Report of a case of retinal and vitreous metastases of a systemic melanoma, possibly arising in the lung, that responded favourably to radiotherapy. CASE REPORT: Retinal and vitreous metastases were demonstrated in a 57-year-old woman during routine follow-up after surgical resection of a melanoma presumed to be a primary pulmonary melanoma. After a 7-week observation period, which confirmed the progressive nature of the intra-ocular lesions, the patient was treated by external beam radiotherapy at a dose of 35 Gy delivered in 14 fractions of 2.5 Gy. Complete disappearance of the vitreous invasion and progressive elimination of the retinal invasion were observed over a period of 9 months. Final visual function was 20/25. REVIEW OF PUBLISHED CASES: A review of the literature identified 28 cases of melanoma with metastases to the retina and vitreous. In almost all of these cases, the primary tumour was a cutaneous melanoma and the mean patient survival following the diagnosis of intra-ocular metastases was 22 months. Retinal metastases, as in the case reported here, present a vascular tropism and tend to develop around veins. These metastases are generally unilateral and may be either solitary or multiple. Tumour invasion of the vitreous occurred by means of isolated cells forming a suspension of aggregates or spherules. Vitreous haemorrhage and irreducible neovascular glaucoma leading to functional impairment, which requires enucleation, were both the most frequent and the most serious complications of these metastases. Treatment is always palliative and is effective in cases with limited retinal and vitreous invasion, as in the case reported here. CONCLUSIONS: Metastatic melanoma in the retina and vitreous is a rare entity and can lead to functional impairment and enucleation because of neovascular glaucoma. As treatment is only effective in cases with limited invasion, systematic screening is recommended in all patients with a metastatic cutaneous melanoma presenting with suggestive ocular symptoms.