A 64 year old Caucasian male was referred in 2005 with a 4 month history of left non-axial proptosis. He had previously undergone local resection of a left uveal melanoma in 1977 followed by plaque radiotherapy for suspected recurrences in 1981 and 1991. Biopsy of a pigmented fornix lesion confirmed recurrence and orbital imaging showed massive retrobulbar orbital extension. Metastasis screening was negative and an extended orbital exenteration was performed with the aim of curative treatment. Histological examination and cytogenetic analysis of the tumour demonstrated low grade characteristics with a favourable prognosis. This case highlights the natural history and treatment of uveal melanoma with late orbital recurrence, the importance of meticulous ocular examination and advances in prognostication of this tumour.