Introduction: Low-grade mucinous neoplasms (LGMNs) of the appendix are rare entities, representing a spectrum of neoplastic disorders characterized by the production of mucin. These neoplasms can lead to the formation of a mucocele, an abnormal dilation of the appendix filled with mucinous material. Appendiceal mucoceles often present nonspecifically, ranging from asymptomatic presentation to mimicking acute appendicitis. Advanced imaging techniques such as CT and MRI play a crucial role in preoperative diagnosis. Surgical resection remains the cornerstone of treatment, with approaches varying based on mucocele size, mucinous dissemination extent, and presence of complications. Case Presentation: A 54-year-old female presented with intermittent lower abdominal pain for one year, gradually worsening over time. Examination revealed a soft, non-tender abdomen with a palpable lump in the right iliac fossa. Imaging showed a distended appendix, suggestive of a mucocele. Exploratory laparotomy confirmed a grossly distended appendix which was excised in toto. Histopathological examination revealed a low-grade appendiceal mucinous neoplasm with extensive calcifications. The patient had an uneventful postoperative course and no remnant malignancy at two-month follow-up. Discussion: Appendiceal tumors are increasingly recognized, with rising incidence reported in recent studies. The classification and terminology of appendiceal tumors has evolved, with the PSOGI Consensus of 2016 providing a comprehensive framework. Mucocele of the appendix can arise from both benign and malignant causes. Surgical resection, ranging from appendectomy to cytoreductive surgery combined with HIPEC, is essential for management . Accurate diagnosis and appropriate surgical intervention are crucial for improving patient outcomes and minimizing recurrence risk. Conclusion: The rising incidence of malignant appendiceal Neoplasms necessitates a refined approach to classification, diagnosis, and management. Surgical resection remains pivotal, tailored to individual patient characteristics and disease extent, with advanced techniques improving outcomes.