We describe a patient with multiple fibrofolliculomas (FF), tricodiscomas (TD) and acrochordons (Birt-Hogg-Dubé) associated with intestinal polyps. One of the polyps presented histological features of marked dysplasia. This association may not be fortuitous and suggests that patients with multiple hamartomas of the perifollicular connective tissue should be examined periodically for intestinal polyps before malignancy develops.