Perioperative management in patients suffering from systemic mastocytosis is challenging. Most recommendations regarding anesthetic management in these patients are based on clinical reports, and there are controversies about the use of rocuronium and sugammadex. We present a case report of a patient with systemic mastocytosis who was given sugammadex for rocuronium reversal. Tryptase levels were monitored during the first postoperative 24 h, without evidence of elevation. We also performed a systematic review to provide an overview of current evidence regarding the safety of using sugammadex in patients suffering from systemic mastocytosis. The search strategy included PubMed and Google Scholar. All studies published up to and including January 2021 concerning anesthetic management in systemic mastocytosis were included. Of the 122 articles located, 9 articles were included: 2 reviews and 7 case reports. Data from reviewed studies confirm that sugammadex can safely be administered in patients suffering from systemic mastocytosis.