Rhabdomyosarcoma, the most common soft tissue sarcoma of children, carried a 10-15% survival rate in the late 1960s. Since then, better understanding of biology and pathology of the tumor and the judicious use of chemotherapy and radiation has improved the prognosis drastically. In addition instead of extirpative surgery, organ salvage is now feasible in many of these children. In this article the authors review current information regarding pathology, diagnosis and treatment of rhabdomyosarcoma.