A two-year, 2-month old girl with Ehlers-Danlos syndrome Type VII is described. The patient presented with bleeding after tooth brushing, and exhibited microdontia and yellow discoloration of teeth. She had carious teeth, but did not exhibit periodontal disease, poor wound healing after extraction, radiographic evidences of pulp stones, or malformed roots. Microscopic examination of a decalcified section of an extracted tooth stained with H & E demonstrated some inclusions in the dentin, around which collagen was either missing or scanty, as confirmed by counterstaining with van Gieson's solution. The sections of pulp stained with van Gieson's solution showed an abnormal pattern of fibrous tissue. Furthermore, the radiopacity of the dentin of this patient was significantly higher than that of the control dentin.