We present the case of a 22-year-old woman with Klippel-Trenaunay-Weber syndrome who presented with clinical signs and symptoms of gastrointestinal obstruction. Ultrasonography and magnetic resonance imaging showed massive splenomegaly with multiple cysts replacing the normal parenchyma of the spleen. Histologic study after splenectomy confirmed the diagnosis of cystic lymphangioma of the spleen, which is an exceptional manifestation of Klippel-Trenaunay-Weber syndrome.