Keratoacanthoma centrifugum marginatum (KCM) is a rare distinct variant of keratoacanthoma. Based on three personal observations and a review of the literature, the authors describe the clinical and histological features of this neoplasm. Clinically KCM is characterized by the lack of a tendency for spontaneous remission and by continuous centrifugal spread. Histologically there is a subclinical, iceberg-like growth pattern. Like keratoacanthoma, KCM is a highly differentiated, biologically benign, non-metastasizing tumour. The treatment of choice is early excision of the tumour.