Face, limb, and oral abnormalities are hallmarks of Papillon-Léage-Psaume Syndrome. It is inherited in a prenatally lethal manner that is X linked dominant (XLD). We reported a case of 13-year-old male who was prima facie diagnosed with aforementioned syndrome .Early diagnosis from an odontogenic perspective can lessen the physical and dental abnormalities that may develop as a result of this condition, so it deserves the attention of paediatric dentists