A pigmented choroidal lesion was observed 41 years prior to enucleation. The patient, with a clinical diagnosis of malignant melanoma in an eye with good visual function, was followed up for the last 25 years by one of the authors. Sudden growth of the lesion and loss of vision led to enucleation and allowed histologic confirmation of the diagnosis of malignant melanoma. A pigmented iris lesion in the same eye was also present for 25 years and showed no notable growth under observation. The patient died of metastatic disease approximately one year after enucleation.