Right ventricular myxoma is very rare, especially its originating from the right ventricular outflow tract (RVOT) and extending to the main pulmonary artery. Here, we report a case of a giant RVOT myxoma, indistinguishable from pulmonary embolism (PE). Although the myxoma is a candidate for urgent surgery, this case satisfied diagnostic criteria for PE and had no indication for intervention, according to the guideline. The strategy for this mass can be completely different, depending on the diagnosis. Surgical extraction was selected because of atypical clinical course, findings, and nagging debut for neoplasm. Then it made hemodynamic status stable by releasing RVOT obstruction and allowed to reveal the diagnosis as myxoma histopathologically.