Several dental conditions are important signals for the presence of certain major diseases. When a patient presents to you with peculiar complaints, it is very important that you, as his/her dentist, consider the possibility of more complex and rare diseases, in order to enable the correct and accurate treatment. Here, we present a similar case of a rare dental syndrome. Case presentation A 27-year-old female patient presented to a dentist complaining of gum enlargement for 7 months. History Her parent’s marriage was non-consanguineous. However, her younger sister demonstrated the same features as hers.

She had two natal teeth in her lower front lower jaw during birth. She had undergone surgical removal of her extra digit on the right-hand ulnar side. Extraoral examination Figure 1. Convex face of the patient Figure 2. Hexadactyly of left hand The patient’s height was 118 cm with a…