Rhabdomyosarcoma (RMS) represents the most common orbital malignancy in children; patients with this disease often present to the ophthalmologist. Because current therapeutic regimens offer an excellent chance for curing isolated orbital disease, prompt diagnosis and treatment are essential. Four decades ago, the standard of care for these tumors was exenteration. Even with exenteration, survival was poor. Much of the success in reducing the morbidity and mortality over the past three decades has been through the collaborative efforts of the Intergroup Rhabdomyosarcoma Studies (IRS) formulated in the 1970s. Treatment of RMS with multiple modalities has transformed the dismal 25% 3-year life expectancy of the 1960s to overall survival (OS) rates of higher than 90% today. With such success, clinicians now have the opportunity to focus on minimizing the serious late sequelae of aggressive therapy.