Rhabdomyosarcoma (RMS) is well known as a pediatric disease. Most of the knowledge, like biology, genetics, and treatments of this disease, comes from studies done in that age group. The two subtypes of RMS, embryonic RMS and alveolar RMS, that affect mainly the pediatric population are well described in the literature and that has had an impact on the improvement in overall survival during the past 20 years. RMS in the adult population has a low incidence, therefor the study of RMS in this group is challenging. Pleomorphic RMS is the subtype that mainly affects adults and its biology and genetics are not yet completely understood and described. The risk factors for this tumor and the differences among adults and children is also poorly understood. The treatments for adults that have RMS are not standardized having an impact on the overall survival. Pleomorphic RMS has, compared to other adult sarcomas, poor overall survival. Adult patients with RMS have poor prognosis. The standardization of treatments for the adult population is necessary as maybe new treatments for this specific group. There are new treatment options that are being studied mostly in pediatrics and young adults. Immunotherapy is currently proposed as an important treatment possibility including different techniques like vaccination, antigen-mediated therapy, and immune checkpoints. Even if we have a better understanding of RMS, there are still unanswered questions. The improvements seen in the pediatric population are encouraging, but there is still the need to enhance better therapies for adults with RMS.