The presence of extraocular extension of uveal melanoma is a known prognostic factor that significantly affects patient survival and the risk of orbital recurrence. Interest in this uncommon event in patients with uveal melanoma has been renewed owing to recent reports of extraocular growth after conservative treatment. Ultrasonography and magnetic resonance imaging seem to be the most sensitive imaging techniques to detect even small degrees of extraocular extension. However, some cases are identified at surgery during enucleation or brachytherapy. Although there is a lack of consensus as to the optimal therapeutic management of orbital invasion in uveal melanoma, the current treatment options include isolated resection and modified enucleation or exenteration combined with radiotherapy or other forms of adjuvant therapy.