Background: The three defining characteristics of systemic sclerosis (SSc), a chronic connective tissue condition, are fibrosis of the internal organs, joints as well skin, autoimmunity, and vasculopathy. New therapeutic targets are urgently required because there is currently no medication that alters the fibrosis components. This review looks at the state of therapies now and new medicines. Objective: This review article aimed to assessment of updated treatment modalities of systemic sclerosis. Methods: We searched PubMed, Google Scholar, and Science Direct for information on Systemic Sclerosis with its treatment. However, only the most current or comprehensive studies from April 2007 to May 2021 were considered. The authors also assessed references from pertinent literature. Documents in languages other than English have been disregarded since there aren't enough resources for translation. Unpublished manuscripts, oral presentations, conference abstracts, and dissertations were examples of papers that weren't considered to be serious scientific research. Conclusion:The development of progressive pharmacological therapy strategies in conjunction with nonpharmacological procedures is the foundation for the treatment of SSc and is established on the regular and routine examination of any possible organ injury. Based on unique traits of the patient and the SSc, several advancements have been accomplished, particularly in the fields of targeted treatments and customized medicine.