Rhabdomyosarcoma (RMS) is a highly malignant tumor in which the tissue of origin is pluripotent mesenchyme. It is the most common soft tissue sarcoma of the head and neck in childhood and comprises 4% of all pediatric malignancies, with 10% of all cases occurring in the orbit. Most of RMS occur in the first decade of life; however, it has been reported from birth to the eighth decade. Recently, we experienced a rare case of a 33-year-old man with orbital RMS; therefore, we present this case with a review of literature.
