Abstract: Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic ocular syndrome characterised by diffuse uveal thickening and proliferation of benign melanocytes which leads to progressive loss of vison bilaterally. The pathogenesis of BDUMP is unclear. The typical clinical findings is a giraffe-like pattern with multiple scattered oval or round subretinal greyish patchy lesions separated by polygonal orange pigmentation. Multimodal imaging and immunologic investigations facilitate early diagnosis of BDUMP. Plasmapheresis with simultaneous treatment of the underlying malignancy is the most effective treatment. (Int Rev Ophthalmol, 2021, 45: 158-164)

Key words: bilateral diffuse uveal melanocytic proliferation, paraneoplastic syndrome, uveal/immunology