We present the case of a 56-year-old man who developed a neoplasm of epithelioid histology in his anophthalmic left orbit 21 years after he underwent enucleation for a spindle cell iris melanoma. The recurrent tumour was managed by orbital exenteration. Neither further recurrence nor metastasis was diagnosed over a 5-year follow-up period. This case, along with five other similar cases in the literature,1–3 emphasises the importance of long-term follow-up after treatment of iris melanoma.
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