Mucocele of the appendix, a nonspecific and descriptive term for an abnormal mucous accumulation within the appendiceal lumen, regardless of the underlying cause, is a rare clinical disease that is not usual as a consideration in the differential diagnosis of right lower quadrant lesions. The reported prevalence of mucocele of the appendix in appendectomy specimens is 0.2-0.3 percent. Hematuria due to mucocele of the appendix is extremely rare. Up to now, only few cases of such condition have been recorded in the literature. We describe a woman who experienced intermittent episodes of right lower quadrant pain and hematuria. Abdominal exploration incidentally displayed mucocele of the appendix. No evidence of other lesions was found. The patient was still in good health after operation. This case highlights mucocele of the appendix as a consideration in the differential diagnosis of right lower quadrant pain with hematuria.