Epstein-Barr virus infection has been linked to the development of smooth muscle tumors in immunocompromised patients with organ transplants and acquired immunodeficiency syndrome. A 52-year-old female recipient of a renal transplant presented with enlarging masses of the left iris. Incisional biopsy of the mass revealed a smooth muscle tumor of the iris. Epstein-Barr virus infection was confirmed by in situ hybridization for Epstein-Barr virus-encoded, small RNA in tumor cells. Eight months after total iridectomy the patient was free of disease. Although the prognosis and classification of Epstein-Barr virus-associated smooth muscle tumors are controversial, mortalities caused by these tumors are rare.