A patient with multiple deformities of ectodermal and mesodermal origin has been reported. The findings of pterygium of the neck, deformities of the extremities, hypoplasia of the mandible, retarded growth, lymphangiectatic oedema, cutis laxa, deformity of the ear, ocular hypertelorism, high-arched palate amid mushroom epiphyses are typical of the status Ullrich bilateralis. An additional finding, which differs from the usual description, was the hyperplasia of the mammary glands.