Wilms' tumor (WT) is frequently associated to congenital malformations, i.e. aniridia (0.8%), hemihypertrophy (2.5%), and genitourinary malformations. Ear malformations have been suggested to be a sign of genitourinary malformations. On the other hand WT cases associated to ichthyosis has never been reported. The authors present a case of Wilms' tumor (WT) associated to ear malformations, ichthyosis and polycythemia, and suggest that such a malformation pattern might represent a new syndrome.