In the present study, a peculiar fibromatosis cutis in two siblings has been reported, the dermatosis being characterized by innumerable perifollicular fibromas on face, neck and trunk as well as multiple fibromata pendulantia. Since the father allegedly had skin lesions resembling those of his two affected children, an inherited condition is assumed for the disease which manifests itself rather late in age. In the female patient, several adenomatous colon polyps were found, one transformed into an incipient carcinoma. Since the clinical and dermatohistological features are anything but typical of Gardner's syndrome and, in particular, hamartomalike fibromas of the perifollicular hair sheath are not constituents of its well-known skin tumour complex, we have discussed in detail the possibility of a peculiar cutaneo-intestinal syndrome hitherto unknown.