Of these genes, deletion or mutation of HNF1B has been associated with renal cystic disease, diabetes mellitus, and liver, pancreas, and female genital tract abnormalities [2]. LHX1 is a candidate gene for the neurocognitive phenotype found in 17q12 deletion and is also expressed in the developing kidneys [1]. The gene discussed is LHX1; the disease is cystic kidney disease.