We describe herein a 4-year-old girl with primary rhabdomyosarcoma of the right iris. Malignant cells were aspirated from the aqueous humor, and the eye was enucleated 7 months after the tumor appeared. To our knowledge, only two cases of primary rhabdomyosarcoma of the iris have been reported. Both of these patients received radiation therapy prior to enucleation. © 1991, American Medical Association. All rights reserved.