A 14-year-old female with ulcerative colitis developed right anterior cervical pain and high fever. Cervical contrast-enhanced computed tomography (CE-CT) showed a wall thickness of the right common carotid artery which suggested aortitis. Her pulmonary angiography demonstrated a narrowing of the pulmonary arteries and she was diagnosed as having Takayasu's disease associated with ulcerative colitis. HLA analysis showed Bw52 and DR2 haplotype, which is frequently found in patients with Takayasu's disease associated with ulcerative colitis.