Recently, our institution reported on a rare primary retroperitoneal mucinous cystadenocarcinoma which was only the second type of this kind of tumor ever reported in a male patient. To our knowledge, we report the first male case of a primary mucinous cystadenoma presenting as an enlarging scrotal mass. These lesions are extremely rare and represent only 0.3% of all appendiceal specimens. Because the number of these tumors remains limited, proven treatment regimens and the necessary follow-up have yet to be elucidated. We hope to provide further insight in the monitoring and treatment of these tumors.