Diffuse bilateral uveal melanocyte proliferation is a rare paraneoplastic syndrome causing severe visual impairment. We report the case of a 58 year old man with progressive visual loss due to multiple retinal pigment epithelial lesions with a serous detachment of the posterior pole in both eyes. Visual acuity improved with systemic corticoids. Two years later, he again experienced visual loss. Meanwhile this patient had undergone a resection of a large cell anaplastic lung carcinoma. The fundus appeared unchanged except for the appearance of multiple naevoid lesions. The choroid was diffusely thickened. Visual acuity improved again with systemic corticoids. This case is quite exceptional as the visual acuity was well preserved during a follow up of more than two years. The case is compared with those published previously and the differential diagnosis is discussed.