Uveal melanoma (UM) is the most common primary intraocular malignancy in adults. At initial staging, distant metastasis is recognized in fewer than 5% of patients, and is generally associated with a poor prognosis. Published guidelines suggest that distant metastasis is not a contraindication to primary tumor therapy, however, it is unclear whether this influences the natural history of the disease. To our knowledge, no studies have investigated the outcomes of patients who present with metastatic UM at initial staging, nor have any studies examined the impact of primary tumor therapy, such as brachytherapy or enucleation. We hypothesized that primary tumor therapy might prevent symptoms (ocular pain), but would not affect overall survival. We performed an institutional review board-approved retrospective study of UM patients found to have metastatic disease at initial staging, within 6 months of initial diagnosis. We recorded patient, cancer and treatment characteristics. We analyzed the development of ocular pain and overall survival in patients treated with and without primary tumor therapy. Cohort characteristics were summarized descriptively, while proportions were analyzed by Fisher’s exact test, and survival was compared using log-rank tests. Of 1238 UM patients seen at our center between 2001 and 2017, 20 (1.6%) had metastasis discovered at a median of 17 days after initial diagnosis; 2 patients presented with liver metastases and were subsequently found to have UM. Median age was 65 years (range, 22-85) and 50% were women. Sixty five percent of patients had a Collaborative Ocular Melanoma Study (COMS) large primary tumor with median base diameter of 18 mm (range, 9.1-35). Metastases were detected by liver MRI in 50% of cases, confirmed by biopsy in 95% of cases, and were a median 2.3 cm (range, 0.9-7.3) in size. Eye pain developed 3-6 months after diagnosis in 3 of 9 patients who did not receive upfront primary tumor therapy, while it did not occur in any of the 11 patients who received upfront primary tumor therapy (brachytherapy [n=6], enucleation [n=5], p=0.07). Primary tumor therapy effectively palliated pain in all cases (brachytherapy [n=1], enucleation [n=2]). All patients treated with brachytherapy retained vision of the eye. Median overall survival was 12.2 months (range, 4.4 – 21.1). Median overall survival was 11.5, 14.3, and 10.8 months for patients that did not receive primary tumor therapy, those who received brachytherapy and enucleation, respectively (p=NS). Patients presenting with metastatic UM at initial staging experience limited survival. Primary tumor therapy does not seem to influence survival, but may help prevent ophthalmic symptoms. Palliative therapy at the time of symptom development seems effective and may be the most appropriate strategy in this patient population.