&NA;Ecthyma gangrenosum is a cutaneous gangrenous disorder which usually followsPseudomona aeruginosainfection and is found mainly in immunosuppressed children. We describe a case of a five‐year‐old female with leukemia with a severe perineal ecthyma gangrenosum resulting in a cloaca‐like deformity. One year later a perineoplasty with puborectalis interposition and overlapping external anal sphincteroplasty was successfully performed, achieving satisfactory continence.