This scoping review aims to explore the relationship between cardiac myxomas (CMs) and paraneoplastic syndromes (PS). CMs are the most common tumors in the heart, with the majority located in the left atrium, and are often associated with a triad of obstructive, embolic, and constitutional symptoms. However, they can also present with unrelated symptoms that are part of a PS. This study performed a thorough literature search of 11 databases and included 12 papers in the final review. All of the patients were diagnosed with atrial myxoma, which initially presented as a PS. Surgery was the curative measure in all cases and resulted in remission in every case, with patients reporting resolution of symptoms at follow-up. The majority of patients in the study were female, with comorbid rheumatologic conditions often present. This study highlights the heterogeneity of presentations of CMs and their associated PS.