Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome of vascular malformations and soft tissue and bone hypertrophy. Vascular malformations can affect multiple organ systems. Involvement of the gastrointestinal (GI) tract is uncommon in KTS, but it can be a source of life-threatening bleeding. We report a case of a 32-year-old male with a known diagnosis of KTS who presented with a life-threatening rectal bleeding and was treated with proctosigmoidectomy and massive blood products transfusion. He expired after a long hospitalization. We then review the literature on KTS and management of some of its complications. 1. Introduction Klippel-Trenaunay syndrome (KTS) was first described by two French physicians, Klippel and Trenaunay, in 1900 [1]. The term describes a rare congenital syndrome of venous, lymphatic, and capillary malformations and soft tissue and bone hypertrophy of usually one limb [2] (lower limb is involved more frequently with extension to the trunk). It seems that the right lower limb is affected more frequently than the left lower limb. Patients can be diagnosed with KTS with only one or more of the abovementioned features since patient might not have all the features [3, 4]. Vascular malformations have been reported to affect the gastrointestinal (GI) tract, liver, spleen, and heart and follow a progressive course [2, 5]. Left-sided inferior vena cava (IVC), secondary to persistence of the left and regression of the right supracardinal vein, has been reported [5, 6]. Vascular malformations can also affect genitourinary tract (kidney, bladder, penis, scrotum, vagina, and vulva) and manifest as intrapelvic or retroperitoneal vascular masses [7]. In this paper, we present a case of a 32-year-old male with KTS who presented with a life-threatening rectal bleeding. 2. Case Presentation A 32-year-old male presented with a sudden onset of massive rectal bleeding. He had a history of KTS since childhood. He had multiple admissions for bleeding, presenting mainly as hematuria that started at the age of 11. He also had one episode of self-limited rectal bleeding four years prior to this presentation. His medications were multivitamins with iron supplement. At the time of admission, physical examination was remarkable for blood pressure of 55/29？mm？Hg, HR of 135 per minute, respiratory rate of 22 per minute, and normal temperature. He appeared pale and diaphoretic. Cardiopulmonary examination was unremarkable except for tachycardia and tachypnea. His abdomen was soft and nontender, with active red blood oozing from his rectum. He was
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