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To report a case of bilateral diffuse uveal melanocytic proliferation associated with renal carcinoma and to illustrate the importance of ancillary examinations to early diagnosis and treatment.Clinical case report.A 56-year-old man reported a 3-day history of visual impairment and scotoma in the right eye. An ophthalmoscopic examination, visual field test, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, optical coherence tomography, and systemic evaluation were performed.Fundus examination showed multiple nevus-like uveal pigmented lesions bilaterally. Optical coherence tomography showed a subfoveal serous retinal detachment and focal loss of the retinal pigment epithelium with adjacent areas of retinal pigment epithelial thickening in the right eye, explaining the scotoma on the visual field examination. Indocyanine green angiography showed multiple round areas of hypofluorescence corresponding to the nevus-like pigmented tumors seen on funduscopy, and retinal pigment epithelium damage corresponding to hypoautofluorescence on fundus autofluorescence imaging and window defects points seen on fluorescein angiography bilaterally. After bilateral diffuse uveal melanocytic proliferation diagnosis, a systemic workup showed clear cell carcinoma in the left kidney. Owing to the tumoral size, chemotherapy was administered.Renal carcinoma associated with bilateral diffuse uveal melanocytic proliferation is rare, and the patterns observed in the ancillary examinations, including indocyanine green angiography, are useful for early-stage diagnosis and immediate referral for systemic investigation and treatment.