ABSTRACT:The mucocele of the Appendix is an uncommon cystic lesion characterised by distension of the appendiceal lumen with mucus. The incidence ranges from 0.3% to 0.7% of all appendectomied specimens. OBJECTIVE: We report here the demographic, clinical, radiologic, histopathologic, preoperative and postoperative findings and outcome of patients of mucocele of the appendix diagnosed on histopathologic examination. METHODS: We retrospectively analysed the hospital records of all the patients whose appendectomy was done and had a histopathological diagnosis of mucocele of the appendix. RESULTS: A total of 885 patients with the preoperative diagnosis of appendicitis were admitted and surgically treated in our centre from April'2004 to September'2012 and 13 (1.47%) patients were diagnosed to have mucocele of the appendix on histopathologic examination. Average age of presentation was 53yrs (range 17yrs -85yrs). 6(46.15%) patients were male and 7 (53.85%) were female with M: F ratio 0.86:1. The most common presentation was right lower quadrant pain in 7 (53.85%) patients. 2(15.38) patients presented with generalised abdominal pain, distension of abdomen and loss of appetite while 4(30.77%) patients were asymptomatic. In preoperative ultrasonographic examination, appendiceal cystic masses were diagnosed in 7 (53.85%) patients. Histopathologic examination revealed simple mucocele in 7 (53.85%) patients, mucinous cystadenoma in 5 (38.46%) and mucinous cystadenocarcinoma in 1(7.69%) patient. Two patients of mucinous cystadenoma were diagnosed with complication of pseudomyxoma peritonei. No mortality was noted in any of the patient postoperatively and on follow up. CONCLUSION: Mucocele of the appendix is a rare disease and is usually diagnosed on histopathologic examination of Appendectomied specimens. As there is potential for malignant transformation and pseudomyxoma peritonei due to rupture of the mucocele, extensive preoperative evaluation and thorough intraoperative gastrointestinal, ovarian & peritoneal examination is required.