A 91/2-year-old boy had clinical and laboratory manifestations of both tumoral calcinosis and pseudoxanthoma elasticum: large periarticular calcified masses, hyperphosphatemia, normocalcemia, calcified blood vessels, and skin lesions of pseudoxanthoma elasticum. A younger sibling had similar, but smaller, calcified periarticular masses and hyperphosphatemia; a maternal aunt was reported to have heterotropic calcifications. This case may represent the coincidental association, hitherto not described, of two rare entities in the same individual, or a widened scope of pseudoxanthoma elasticum; finally, it may represent a new entity of tumoral calcinosis, hyperphosphatemia, and pseudoxanthoma elasticum-like lesions which is familial and in which all the manifestations may not be expressed in every affected individual. © 1968 The C. V. Mosby Company.