Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder, characterized by the triad of vascular malformations (angioma), venous and or lymphatic malformations and asymmetrical disturbed growth of soft tissues and/or bone. Primary rectal linitis is a rare digestive tumor with very poor prognosis. We report the case of a 41-year-old patient with Klippel trenaunay syndrome who was diagnosed with primary rectal linitis.
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