Aim: Starting from a case presentation, this review aims to present literature data on inflammatory fibroid polyps (IFPs) of the small intestine. Methods: Case report and systematic review. A comprehensive systematic review of English literature using PubMed was conducted, based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The used key words were: “inflammatory fibroid polyp” or “Vanek”, including only cases with IFPs localized of the small intestine, published from 1976 to 2019. Results: We present a case of a 38-year old patient with intestinal IFP presenting with acute abdomen due to intussusception diagnosed with ultrasound (US) based on a target sign and visible solid tumor in the small intestine leading to prompt surgical treatment. A diagnosis of IFP was made based on the pathohistological findings. Moreover, a systematic review of small intestine IFPs was conducted which is, to our knowledge, the first comprehensive systematic literature review on this topic. The analysis included 53 case reports or case series concerning 77 cases of small bowel IFPs. The patients were aged from 4 to 75 years (average 45.2), with a female predominance (59.7%). The most common localization was the ileum in 77.9% cases, followed by the jejunum (13%) and the duodenum (6.5%). The most common clinical presentation was abdominal pain due to intussusception (63.6%). Regarding diagnostic methods, computed tomography (CT) was frequently used as primary diagnostic method (26%) followed by exploratory laparotomy (16.9%), endoscopy (7.8%) and US (6.5%). Combination of US and CT contributed to the diagnosis in 9.1% of cases. The majority of cases were treated surgically (92.21%), while only a minority benefited of minimally invasive techniques such as endoscopy. Conclusions: Small bowel IFPs, ones of the least common benign tumors, are characterized by variable clinical signs and symptoms and can potentially lead to serious consequences for the patient.