Appendiceal mucinous neoplasms are an extremely rare group of neoplasms and can range from mucocele to invasive adenocarcinoma. Low-grade appendiceal mucinous neoplasm (LAMN) is an extremely rare form of mucinous neoplasm affecting the appendix. In the majority of patients, it presents with acute abdomen, nausea, vomiting, and change in bowel habits. Pseudomyxoma peritonei is the most dreaded complication. We present a case report of a 35-year-old female who presented to the surgical emergency department of our hospital with complaints of acute abdomen. On examination, a lump was felt in the right iliac fossa, due to which a clinical diagnosis of acute appendicitis with perforation was suspected. However, histopathological examination revealed low-grade appendiceal mucinous neoplasm. Therefore, LAMN should be considered as one of the differentials in patients, who present with acute abdomen and right iliac fossa mass.
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