Digeorge syndrome is also known as velo-cardio-facial syndrome, it is a complex genetic multi-organ disorder occurs due to deletion in 22q11.2 chromosome. Patients usually presented with congenital heart defects, characteristic faces that includes: retrognathisa or micrognathia, low set ears, hypertelorisim. They may also present with ophthalmic, renal, pulmonary and gastrointestinal abnormalities. We report a case of 11 years old male with this type of syndrome and his oral condition.