Abstract: A 2 1/2 ‐year‐old boy with eosinophilic granuloma confined to the frontoparietal bones developed a rectal fissure that progressed to a perianal plaque resembling condyloma latum. Biopsy confirmed the presence of many Langerhans cells, and therefore a diagnosis of eosinophilic granuloma involving the skin was made. Although rare, eosinophilic granuloma can be associated with cutaneous lesions, sometimes isolated and mundane in appearance. It should be considered in the differential diagnosis of perianal lesions.