Ocular fundus abnormalities associated with the Klippel-Trenaunay-Weber syndrome are uncommon and include retinal vascular tortuosity and diffuse choroidal hemangioma. A case involving a young girl with Klippel-Trenaunay-Weber syndrome who had unusual bilateral, exudative, outer retinal vascular masses involving the peripheral fundus in one eye and the foveal area in the other eye is reported. Kidney biopsy for renal insufficiency disclosed abnormal excess mesangial tissue. The fundus lesions appear to represent vascular tumors of the retina that differ clinically from previously reported retinal vascular tumors and Coats disease. The simultaneous retinal and renal involvement suggest that Klippel-Trenaunay-Weber syndrome may be associated with more widespread vascular malformations than previously realized.