Inherited polycystic kidney disease (PKD) in children is a disorder characterized by diffuse cystic involvement of both kidneys, without evidence of dysplasia. Both forms, autosomal recessive (ARPKD) and autosomal dominant (ADPKD) have considerable overlap in clinical presentation and radiographic features in the pediatric population. At present, a prenatal diagnosis is possible with ultrasound examination. A brief review of the literature is here reported. The knowledge of pathophysiological and clinical data is requested, since PKD represents a major cause of renal failure in pediatrics.