Mucocele of the appendix is an uncommon disorder that is often asymptomatic, but can present similarly to acute appendicitis. Timely diagnosis and treatment is imperative due to the many complications that can result from the mucocele, such as perforation. Appendiceal mucoceles (AM) were previously thought to be either benign or malignant; however, a different pathological classification of AM is currently favored. Also, only a few cases of volvulus of a benign AM have been reported. Here, we present the first reported case of a low-grade appendiceal mucinous neoplasm resulting in a volvulus of the cecum.