To report a case of melanocytoma originating from the iris observed for the first time in Korea.

A 53-year-old female with an unexpected iris mass was referred to our clinic. A round, 2.5 mm × 3.5 mm-sized iris mass was found on slit lamp examination in the 12 o'clock area of the patient's left eye. The mass was densely pigmented and had a smooth surface. Gonioscopy showed the mass had reached the peripheral cornea frontward and the lens backward. An excisional biopsy was performed for diagnosis. After the operation, a gonioscopic examination showed an intact ciliary body behind the surgical margin of the iris. A melancytoma of the iris was observed on subsequent histopathological examination. The patient has remained symptom-free with no iris mass recurrence since the operation.

A few cases of iris melanocytomas have been reported worldwide but not in Korea. We confirmed a case of melanocytoma originating from the iris for the first time in Korea.