Two cases of the syndrome of webbing of the neck, lymphangiectatic edema, shortness of stature, cubitus valgus and deformities of the toes (symmetric form of the Bonnevie-Ullrich syndrome) are presented. One patient also had elevated urinary gonadotropins and ovarian agenesis. The clinical findings of this syndrome and related conditions are described and the possible mechanisms of pathogenesis are reviewed. The relationship of the Bonnevie-Ullrich syndrome to other clinical syndromes is discussed.