A 68-year-old man diagnosed with choroidal melanoma (CM) in the right eye underwent treatment with episcleral brachytherapy (I 125 ) and transpupillary thermotherapy. Ultrasound, computed tomography, and magnetic resonance imaging were performed and revealed ocular recurrence of CM. Treatment with extended enucleation was performed. Macroscopic and microscopic examinations revealed extraocular extension and malignant cells, respectively. Immunohistochemistry demonstrated tumoral Melan-A and HMB-45 expression. No cytogenic abnormalities were detected with fluorescence in situ hybridization of tumor cells using probes against chromosomes 3q27 and 8q24. The patient underwent adjuvant external beam radiotherapy for treatment of residual tumor tissue. This case represents the first reported case of recurrent CM with no cytogenetic abnormalities and the absence of metastatic disease, despite a number of the poorest prognostic factors.