Mucinous lesions of appendix are a rare clinical entity and may be neoplastic or non-neoplastic. The diagnosis is usual incidental during computed tomography scan or colonoscopy performed for general abdominal symptoms or occasional finding during operation for acute appendicitis. For this reason, initial treatment should be tailored to the situation, aiming at complete resection of the appendix with disease-free margins: this can be achieved by simple appendectomy or more extensive resection. The pathological examination of the specimen is the key to offer the patient a correct and complete treatment, and, if a neoplastic pathology is found, the case should be discussed in multidisciplinary group. We describe three cases with different clinical presentation leading to different surgical treatment: one elective case, in which the diagnosis was suspected preoperatively; and two urgent cases, one mimicking an intussusception and another one presenting as an acute appendicitis.