The pediatric reports of LCKD suggest a congenital component and possible maldevelopment of the kidney, leading to the development of this entity.3,6 Other reports propose a somatic mutation of PKD1 (Polycystin 1, Transient Receptor Potential Channel Interacting), which is a gene responsible for the majority of cases of ADPKD, although LCKD differs in its disease progression.12 Nonetheless, the exact mechanisms underlying cyst formation within localized areas of the kidneys are yet to be fully elucidated. The gene discussed is PKD1; the disease is autosomal dominant polycystic kidney disease.