Lymphedema is extravasation of lymphatic fluid into extracellular spaces and may be primary or secondary. Primary lymphedema may be caused by truncular lymphatic malformation. Long-standing lymphedema may result in various complications due to increased porosity of lymphatics, defective valves, and increased lymphatic pressure. There are few reports of normolipidemic xanthoma in association with lymphedema. We report a case of giant xanthoma associated with primary lymphedema.