The authors report a case of the sign of Leser-Trélat occurring in a patient with primary adenocarcinoma of the rectum. The patient demonstrated the sign several months before the internal malignancy was discovered. Although the majority of patients with the sign have an underlying adenocarcinoma of the gastrointestinal tract, relatively few cases have been reported with the rectum as the primary site. This case demonstrates a classic example of the sign of Leser-Trélat and illustrates its importance as a marker of underlying malignancy.

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