Although acute appendicitis is a common clinical condition in general surgical practice, <1% of them are associated with malignancies. Appendiceal carcinoids make up most of those malignancies and acute appendicitis cases associated with benign cecal neoplasias are very uncommon. In this study, a 25-year-old female patient who presented with distinct acute appendicitis symptoms is reported. The patient was operated on via open technique. Exploration revealed an appendix with advanced edema and hyperemia. While the cecum was observed to be normal, a solid mass of 2.5 cm diameter was palpated in the appendiceal base. Following the ileocecal resection, histopathological examination revealed the mass as a fibromatosis. The goals of this report are to remind health care professionals that some very rare etiologies may be involved in acute appendicitis diagnosis and treatment, and to underscore the place of laparoscopic approach and preoperative computed tomography in this disease.