A case is reported of a 15 years old Chinese female with Larsen syndrome exhibiting many characteristic facial and skeletal features plus hitherto unreported orofacial anomalies including delayed dental development; hypodontia affecting all the permanent canines, second and third molars, maxillary lateral incisors and second premolars; morphological anomalies of the maxillary first premolars and molars; a class 3 skeletal pattern and reduced upper facial height. Cephalometric radiographs showed the skull bones to have an appearance similar to that of osteopetrosis.