Pseudoxanthoma elasticum (PXE) is a heritable disorder of elastic fibers characterized by yellowish, coalescing papules on the flexural area, which is loose and wrinkled. A 35-year-old woman presented cutis laxa-like marked wrinkling and yellowish papules, and a skin biopsy revealed ossification and fragmentation of elastic fibers in the dermis. Ophthalmologic examination revealed a peau d'orange appearance of the retina. She was also found to have a mitral stenosis and hypertension. This is an unusual case of concurrent ossification and cutis laxa-like PXE.