Here we report the case of a 1.5-year-old Iraqi boy who was referred for chemotherapy after left eye enucleation. The patient had a history of left eye leukocoria since 2 months of age. According to history, physical examination and paraclinical work up, he was first diagnosed as a case of retinoblastoma by an ophthalmologist. However, the pathology report favored embryonal rhabdomyosarcoma. In conclusion, a patient with leukocoria should be evaluated carefully for other underlying malignancies.