Medullary sponge kidney and congenital hemihypertrophy

We have presented a case of medullary sponge kidney associated with congenital hemihypertrophy. Evaluation by calcium loading revealed no abnormal handling of this ion. The recognition of this unusual syndrome is important because it appears that other disease processes common to medullary sponge kidney alone or congenital hemihypertrophy alone do not appear with the same frequency when both processes occur simultaneously.