Congenital skin lesions may appear without other abnormalities or in c
ombination with other complex malformations. 86 % of the solitary lesi
ons with a diameter up to 3 cm are found within the scalp. Rarely, mul
tiple lesions are located also at other sites of the integument. Becau
se of the etiological heterogeneity and the possibility of associated
abnormalities in about 44 % of the patients, several diagnostic steps
are required depending on the clinical features of the patient. Based
on representative case reports we want to give a detailed survey of th
e heterogenic spectrum of diseases with congenital skin lesions.