We report on a our generation family with Greig cephalopolysyndactyly syndrome. The clinical variability of the malformations of hand and feet is described. A review of the literature is given, with emphasis on the frequency of clinical sings of the Greig cephalopolsyndactyly syndrome. Polydactyly of the hands and feet are possibly an underestimated features in Greig syndrome. We suggest that radiographs of the hands and feet should be performed in every patient, especially those with broad thumbs. The resemblance of preaxial polydactyly type 4 and crossed polydactyly with Greig syndrome is discussed. These disorder may be allelic.