A case of metastatic tapioca melanoma of the iris in a 12-year-old girl is reported. The patient had heterochromia, a red painful eye, and was treated for iritis with secondary glaucoma. In the course of 5 months iris lesions with the clinical appearance of tapioca pudding developed, and biopsy disclosed a melanoma. The eye was immediately enucleated, and pathological examination showed a melanoma with predominantly epithelioid-type cells which had infiltrated the angle, the posterior chamber, and the surgical wounds. Conjunctival extension was noted 10 months after enucleation, and regional lymph node metastases were found 4 months later. Previously reported cases are reviewed and compared with the present case.