Kocher Debre Semelaigne Syndrome is a rare syndrome with clinicalmanifestation of hypothyroidism associated with pseudomuscularhypertrophy. Very few cases have been reported on kocher-debresemelaigne syndrome in the literature with the incidence reportedto be less than 10%. As per our knowledge; until now, only one caseof oro-facial manifestations has been reported. Here we report onesuch case of Kocher debre semelaigne syndrome in an 11 year oldboy, showing features of macroglossia, multiple impacted teeth,puffed lips and anterior open bite.