Bilateral congenital multicystic dysplasia of the kidney

The clinical and pathological features of congenital multicystic dysplasia of the kidney are reviewed and we report of a patient with bilateral dysplasia who lived longer than do most such patients. When multicystic disease of the kidney is bilateral, it is commonly confused with polycystic disease. Despite the fatal prognosis, differentiation is essential for appropriate genetic counselling since multicystic disease is not a familial occurrence.