Lupus erythematosus (LE) and porphyria can occur together in the same patient and present special problems in diagnosis and management. At least 17 cases of coexisting LE and porphyria have been reported in the literature and this report describes in detail seven additional patients with this combination. The intriguing question is whether the two diseases might be present by some common pathogenic mechanism, but as yet, any etiologic relationship is unclear. The increasing number of reports in the literature indicates that physicians caring for patients with either LE or porphyria should be alert to signs or symptoms suggesting this dual disease process. This is especially important when considering the course of therapy.