Transanal evisceration of the small bowel following spontaneous rectal perforation is a rare condition, potentially lethal if not promptly treated. We present the case of an adult patient, with two previous surgeries due to rectal prolapse. After a strong effort to pass a bowel movement, the patient developed intense abdominal pain and became hemodynamically unstable. Diagnosed with transanal evisceration of the small bowel, the patient required an urgent laparotomy with small bowel reduction and Hartmann procedure. We performed a non-systematic review, with fewer than 100 documented cases of transanal evisceration reported in the literature. Cases can be divided into two main groups: traumatic lesions mainly affecting pediatric patients and spontaneous occurrence. 50% of spontaneous cases associate rectal prolapse, 75% involve an increase in intraabdominal pressure; the majority of cases (70%) occur during adult life (above 40 years old), with no sex-based differences (53% male vs 47% female). Transanal evisceration of the small bowel secondary to spontaneous rectal perforation presents more frequently in adult patients with chronic rectal prolapse and sudden increase of abdominal pressure. Urgent surgery must be performed in all cases to prevent small bowel ischemia. Choice of surgical procedure depends on clinical presentation, patient's comorbidity and age, surgeon's expertise and availability of equipment. We recommend exploratory laparotomy for better assessment of the viability of the small bowel.