The diagnosis of carcinoid tumor of the appendix is rare; the involvement is often limited to the gastrointestinal tract. Extramural involvement of surrounding structures has been reported; however, direct involvement of the ureter has not been reported. We report a case of carcinoid tumor of appendix involving the ureter and causing obstruction. To our knowledge, this is the first case report of such presentation in available literature. A 23-year-old woman who presented with right loin pain and intermittent fever for 1 week duration and found to have gross hydroureteronephrosis on the right side due to a mass compressing the ureter.