The case is described of a woman who suffered since her early childhood from four foci of basalioma and died aged 29 years of metastatic lung involvement. The case appears unique comprising several rare features, e. g. a basalioma in childhood, a metastatic basalioma, and a small-sized metastasizing primary tumour. The disease may be congenital, however, it differs from the typical nevoid basal cell carcinoma syndrome.