Acute arteritis of the aorta and the main pulmonary arteries is unusual in a newborn. This type of vasculitis should be described as a separate entity. There are many differences between this entity and polyarteritis nodosa or Takayasu disease. Moreover, neither Takayasu disease nor polyarteritis nodosa and other forms of classic primary large vessels vasculitis have been reported in a neonate until now. The presence of chronic inflammation, fibrosis, and calcification would suggest that this process had been present before birth.We report a 27-day-old newborn with acute necrotizing aortitis. The feature of this entity is not those of any of the classic entities described in infants or children.