Appendiceal intussusception (AI) is an unusual and rarely reported clinical entity. It was first described by McKidd in a 7-year-old boy in 1858. Since then the literature on it has been confined to a few case reports and very small cases series, in totally about 300 cases being published. Because of variations of clinical symptoms and lack of pathognomonic sign, rarety of the pathology and diversity of causing factors, accurately detection of the presence or absence of appendiceal pathology is difficult. However, imagistic and endoscopic methods may be useful in AI detection and play a large and important role in differential diagnosis. Treatment of AI differs significantly depending on underlying pathological condition, benign or malignant.