Posterior uveal melanoma is the most common primary intraocular malignant tumour and in Sweden some 70-80 new cases present each year. While uveal melanoma is more prevalent in the setting of ocular melanocytosis and neurofibromatosis, there is little conclusive data on the aetiology. Most patients experience a progressive visual field defect and present with a grey or greyish-brown mass of the posterior choroid. Diagnostic procedures include fluorescein angiography, ultrasound and magnetic resonance imaging. In some cases, intraocular biopsy may be required to make a correct diagnosis. Posterior uveal melanomas can usually be managed by any of a number of eye-preserving options like plaque radiotherapy and charged particle irradiation, but eyes containing large tumours are often enucleated. Nearly half of patients with posterior uveal melanoma, and in particular those with large tumours, ultimately succumb to metastatic disease. While most patients with tumour dissemination are treated with systemic chemotherapy possibly combined with interferon, metastatic spread confined to the liver may potentially be managed by intraarterial perfusion chemotherapy or liver resection. However, outcome of patients with systemic disease remains extremely poor with a median survival following detectable tumour dissemination of only two to five months. There are still insufficient data on the impact of various treatments on survival, but a large prospective trial addressing this issue is in progress. The present review summarizes the state-of-the-art knowledge and current management of posterior uveal melanoma from a Swedish perspective.