Mucocele of the appendix is a nonspecific term that is used to describe an appendix abnormally distended with mucus. This may be the result of either neoplastic or non-neopleastic causes and may present like most appendiceal pathology with either mild abdominal pain or life-threatening peritonitis. Urologic manifestations of mucocele of the appendix have rarely been reported. Laparoscopy can be used as a diagnostic tool in equivocal cases. Conversion to laparotomy may be indicated if there is a special concern for the ability to remove the appendix intact or if more extensive resection is warranted, as in malignancy. We here report our experience with a woman presenting with hematuria whose ultimate diagnosis was mucocele of the appendix, and we review the appropriate literature. This case highlights the mucocele as a consideration in the differential diagnosis of appendiceal pathology and serves to remind the surgeon of the importance for careful intact removal of the diseased appendix.