A rare case of a long-term survivor of Menkes' syndrome who was treate
d early and had excision of bilateral axillary skin excess is presente
d. Primary wound healing was observed and skin histology showed fragme
ntation of the elastic fibers. Two years after surgery, the scars appe
ared atrophic with evidence of early recurrence of the skin excess. Th
e relationship between Menkes' syndrome and the other copper-related s
yndromes (X-linked cutis laxa, occipital horn syndrome, and Ehlers-Dan
los syndrome type IX) is also described.