Nodular regenerative hyperplasia (NRH) of the liver is an uncommon disease entity, especially in the pediatricage group. A few cases have been reported in the radiologic literature, but follow-up imaging studies are rare. We describe a case of NRH, diagnosed by ultrasound-guided needle biopsy, in a seven-month-old infant with cri-du-chat syndrome. Initial ultrasound revealed several small hypoechogenic nodules in the liver, but CT and MR failed to demonstrate their presence. Two follow-up sonographic examinations were performed 7 and 20 months later, revealing increases in the size and number of the nodules.