BACKGROUND Choroidal melanoma is the most common primary intraocular tumor in adults. Most primary choroidal melanomas are unilateral and unifocal. Bilateral primary choroidal melanomas are considered to be a rare occurrence. Ocular melanocytosis, especially when it is bilateral, increases the incidence of bilateral primary choroidal melanoma. CASE REPORT Our patient was a 78-year-old man who presented to the Emergency Department with floaters and a reduction in visual acuity in his left eye, with an onset 7 days prior. Upon macroscopic examination, the patient displayed bilateral pigmentation on the sclera, which was consistent with ocular melanocytosis. Fundoscopy revealed a large choroidal melanoma, situated superior and nasally of the posterior pole of the left eye, and a smaller choroidal melanoma, located inferonasally, in the right eye. Ultrasonography, optical coherence tomography, fundus autofluorescence, fundus fluorescein, and indocyanine green angiographies were performed, confirming the diagnosis of simultaneous bilateral primary choroidal melanomas. CONCLUSIONS This was a rare case of bilateral ocular melanocytosis, which increased the probability of bilateral primary choroidal melanoma. To the best of our knowledge, this is the first case in Greece to be reported. This case illustrates the necessity of always examining the fellow eye on initial presentation and over a long follow-up. We should always bear in mind that choroidal melanoma can be a bilateral disease, albeit very rarely.