Pseudoxanthoma elasticum (PXE) is a heritable connective tissue disorder affecting the elastic structures in the body, manifesting with cutaneous, ophthalmologic and cardiovascular findings. The association of PXE and systemic disease is rare, as only six cases have been reported in the literature. We describe herein two cases of coexisting PXE and cutaneous lupus and discuss a possible association. Although a fortuitous association cannot be excluded, the calculated prevalence of the association in our area was much less than in our observation. Given the ocular and vascular complications of PXE, clinicians who treat patients with lupus should be aware of this possible association.