Rhabdomyosarcoma (RMS) is a soft tissue sarcoma (STS) of skeletal muscle phenotype arising from primitive mesenchymal cell, rhabdomyoblast. It is the most common childhood and adolescent STS with slight male predominance, with only 1% of adulthood tumors being RMS [1]. The age distribution is binomial, with higher incidence among 2–4 years of age and 12–16 years. Incidence is lower in the Asian population. Most cases are sporadic; however, some association is found with familial syndromes such as neurofibromatosis type 1, Li-Fraumeni, Beckwith-Wiedemann, Costello, Noonan, and DICER1 syndrome [2].