PURPOSE: We describe a rapidly growing choroidal melanoma that failed to respond to consecutive globe-sparing treatments in an individual with ocular melanocytosis.

DESIGN: Interventional case report.

METHODS: A 53-year-old man with right ocular melanocytosis, decreased vision in the right eye, and right eye pain was referred for suspected choroidal melanoma. The lesion, which measured 2.4 mm × 6.6 mm × 6.0 mm, was treated with transpupillary thermotherapy on two occasions and with iodine-125 (I-125) plaque brachytherapy, both of which failed. Enucleation allowed histologic analysis.

RESULTS: At enucleation, the choroidal melanoma in the right eye measured 13.6 mm × 16.5 mm × 16.9 mm, which demonstrated the most rapid growth and doubling time reported in a tumor of this type thus far. Pathologic analysis classified the tumor as a mixed-cell type.

CONCLUSION: This case reiterates the association between ocular melanocytosis and choroidal melanoma, provides additional evidence that rapid growth and doubling time may be associated with transpupillary thermotherapy and I-125 plaque failure, and highlights the need to follow those tumors closely.