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A 15-year-old boy with Marshall-Smith syndrome presented with increased frequency and urgency of stooling, hematochezia, and rectal pain. A polypoid mass was found at the anorectal junction and excised. Microscopically, the lesion was covered by both squamous and columnar mucosa. It was villiform in configuration with focal ulceration and strands of smooth muscle in the lamina propria. These features are characteristic of an inflammatory cloacogenic polyp, a lesion not previously reported in the pediatric age group. Inflammatory cloacogenic polyp is related to solitary rectal ulcer syndrome and is most likely due to prolapse of the anorectal transition zone. Although rare in this age group, solitary rectal ulcer and its variants should be considered in the differential diagnosis of anorectal and rectal lesions in the pediatric patient.