ABSTRACT
Medullary cystic disease (MCD) and familial juvenile nephronophthisis are inherited diseases characterized by development of small cysts at the cortico- medullary borders of the kidneys. Both diseases consist of microscopic or gross cysts of the renal medullae associated with atrophy of nephrons, anemia, renal insufficiency, polyuria, and salt-wasting. We report a 27-year-old pregnant female patient with MCD which caused acute renal failure progressing to end stage renal disease during the second trimester. Hypertension, oliguria, preeclampsia, placenta abruptio, and disseminated intravascular coagulation were also accompanied. Radiologically, abdominal ultrasonography and computed tomogram showed many cysts confined only to the renal medullae, which are typical findings consistent with MCD. In spite of conservative therapy, her renal function deteriorated rapidly and uremic symptoms including pulmonary edema were aggravated. She was started on hemodialysis and she has received regular hemodialysis without recovery of renal function.