INTRODUCTION: Mucocele of appendix is a rare clinical entity (0.2-0.3% of appendectomy specimens) with obstruction to drainage of mucus from the appendicular lumen. Rarely, does it manifest as bowel obstruction owing to intussusception of mucocele of appendix (0.01%). Diagnosis of mucocele or its intussusception is radiological, can't differentiate if the cause of obstruction is a benign or a malignant process. CASE DESCRIPTION/METHODS: A 40 year female presented with complaints of right lower quadrant pain since a few days, was dull, aching, intermittent, associated with nausea without any obvious aggravating or relieving factors and history of trauma to the abdomen or previous surgery. Abdominal examination revealed tenderness in right lower quadrant without any guarding or rigidity and lump could be felt in the same quadrant. On auscultation sluggish bowel movement. An abdominal CT: intussusception of mucocele of appendix of 4.5 × 3.1 × 2.5 cm. Open appendectomy was planned to avoid accidental spillage of contents and resected margins and regional nodes were negative for malignancy as per histopathology. The postoperative period was uneventful. DISCUSSION: A Mucocele of appendix (MOA) is an abnormal accumulation of mucus in appendix. A cystadenoma, mucosal hyperplasia, retention cyst or mucinous cystadenocarcinoma may be the cause of this obstruction. Multi-detector CT is imaging of choice, classic appearance of a well circumscribed, fluid-filled, distended appendix. Presence of mural calcification differentiate from appendicular abscess. Further, an intussusception of MOA has been likened to a cup & ball pattern. However, features like enhancing mural nodules within the mucocele/size >2 cm point towards a malignant cause. Definitive management is surgery, a mucocele with compromised base, >2 cm require a primary typhlectomy, otherwise a primary appendectomy suffices. Hence, open surgery is preferred over laparoscopic to prevent spillage. Positive margins and/or lymph node involvement translates into right hemicolectomy. A cystadenoma is followed up like an adenomatous polyp while follow up similar to colonic carcinoma is employed in cases of cystadenocarcinoma of appendix.