Various extraintestinal manifestations may occur in juvenile polyposis, but
hereditary hemorrhagic telangiectasia has rarely been reported in this typ
e of polyposis. The authors treated a 14-year-old girl with rectal bleeding
and anemia who had multiple polyps of the colorectum. Large polyps were re
moved and histologically diagnosed as juvenile polyps. She was later diagno
sed as having telangiectasia of the skin, and arteriovenous malformations i
n the lung and in the liver, all of which were compatible with the diagnosi
s of hereditary hemorrhagic telangiectasia. At age 32, she had multiple ulc
ers in the ileum and in the colon. The coexistence of juvenile polyposis an
d hereditary hemorrhagic telangiectasia may be a clue for the understanding
of the histopathogenesis of juvenile polyposis.