A 56-year-old man presented with recalcitrant uveitis with anterior chamber and vitreous cells. He developed small scattered lesions in the macular areas of both eyes, which disappeared in the right eye and evolved to multifocal, discrete, punched-out lesions resembling birdshot retinochoroidopathy in the left eye. Ocular reticulum cell sarcoma (RCS) was suspected but was not confirmed by diagnostic vitrectomy. The patient died 3 years later and was found to have RCS with central nervous system and ocular involvement. The occurrence of tumor cells under the retinal pigment was the apparent cause of the multifocal lesions that disappeared in the right eye and that led to discrete punched-out lesions with no scarring in the left eye.