Delleman syndrome is a rare disorder characterised by orbital cysts, micro/
anophthalmia, malformations of the central nervous system, focal aplasia cu
tis, and multiple skin appendages (oculocerebrocutaneous syndrome). Althoug
h cutaneous findings provide the main clues for the diagnosis, the syndrome
has received little attention in the dermatological literature. A new case
of oculocerebrocutaneous syndrome with predominant and typical cutaneous i
nvolvement is reported.