Vasculopathy is a not well-described presentation of neurofibromatosis type 1 in the dermatologic literature. We report a fatal case of neurofibromatosis vasculopathy in a 28-year-old woman who presented with skin ulcers and livedo racemosa; extensive gastrointestinal ulcers; and infarction of the bowel, liver, and spleen. Being alert to this manifestation may avoid unnecessary delay in diagnosis and enable, whenever possible, appropriate therapy.