We report on a patient with Mu-librey nanism (MIM 253250) who at age 18 months hada Wilms tumor removed. The boy was diagnosed as Mulibrey nanism at age 6 years on the basis of short stature, progressive growth retardation, abnormalform of face with high prominent forehead, hypertelorism, prominent eyes, small triangular chin, hepatomegaly, pericardic constriction, and cryptorchidism.