LAUSR

Highlights • The rare Nievergelt syndrome (NS) is the most severe form of mesomelic dysplasia and is characterized by disproportionate shortness of the limbs.• The primary phenotypic characteristics include a rhomboid-shaped tibia and overgrown fibula with bony protuberances and skin dimples.• These finger-like projections and concomitant rhomboid-shaped tibiae and fibulae should be kept in mind for the NS.• The limb lengthening and deformity corrections using an external fixator could be considered in the orthopedic treatment of the NS.• A preoperative evaluation of the vascular status could prevent several postoperative complications.