Conjunctival myxoma is an uncommon benign tumor that may develop as a localized disease or as part of the Carney complex, in which case it can cause significant morbidity and mortality. Conjunctival myxomas can mimic other pure or mixed tumors and are often overlooked in clinical practice. Histopathological studies are essential for diagnosis; however, controversy regarding their pathological and immunohistochemical features makes the diagnostic process more difficult. Because of the importance of correctly classifying these conjunctival tumors, as well as recognizing when a systematic screening is required owing to the possible association with life-threatening diseases, we comprehensively evaluate the literature on this rare entity based on a systematic approach.