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To present a case of medullary sponge kidney (Cacchi-Ricci disease) in a 33-years-old woman with severe hypertension with special reference to the ultrasound findings that permitted early diagnosis of this rare condition.The most relevant clinical features and ultrasound findings in a patient with Cacchi-Ricci disease are described.We describe here a 33-years-old Moldavian woman with negative family history for hypertension who presented to the physician with severe hypertension, established in a couple of months after childbirth, and resistant to usual treatment with angiotensine conversion enzyme inhibitors. She also had concomitant features of distal renal tubular acidosis (alkaline urine, hypercalciuria). Extensive diagnostic workup was undertaken to find secondary causes for it. The most informative proved to be renal ultrasound which revealed bilateral increased medullary echogenicity, highly suggestive for MSK.Medullary sponge kidney disease might be responsible for secondary hypertension, and simple kidney ultrasound increase the probability of early diagnosis of this rare condition.