Intestinal spirochetosis (IS) is an unusual infection in children, one with no standard therapeutic options. This article reports the findings on 5 new cases in conjunction with a 20-year review of the pediatric literature. The diagnosis of IS in children requires a high degree of suspicion by the physician, as many cases present with abdominal pain, chronic diarrhea, and/or hematochezia associated with a normal endoscopic examination. Silver stains (Dieterle or Whartin-Starry) are the preferred confirmatory stains on tissue sections. Giemsa (Diff-Quik) and periodic acid-Schiff stains may also be of value. Current literature favors the use of metronidazole in adult patients with IS, yet little information is available regarding treatment options in pediatric cases. This review indicates that a macrolide antibiotic with or without metronidazole may represent the best therapeutic choice for children. Further investigations are needed to determine the correlation between IS and coexisting gastrointestinal diseases and/or immunodeficiencies.