[A woman with iris heterochromia]. / Een vrouw met irisheterochromie.

A 20-year-old woman with congenital iris heterochromia presented with loss of vision of her right eye. We made de diagnosis of a large 'uvea melanoma' and enucleated the eye. Pathological examination showed an underlying oculodermal melanocytosis (ODM). The life-time risk of uveal melanoma in the general population is 0.7:100,000, but 1:400 in patients with ODM. Therefore, annual fundoscopy is recommended in these patients.