Pseudoxanthoma elasticum (PXE) is a rare hereditary disease whose basic abnormality is degeneration of elastic tissue, with calcification of the tonaca media of the arteries, and typical yellowish papules of the skin. We report the sonographic findings observed in a young patient with PXE at the level of the kidneys: fine hyperechogenic spots suggesting small calcifications were diffuse at the corticomedullary junction; however, normal waveforms were obtained at the level of intraparenchymal renal vessels. The presence of this structural pattern in a young patient with dermatologic abnormalities should lead to the consideration of PXE in the differential diagnosis list.