Provision of adequate nutrition is a cornerstone of the management of infants and very young children with chronic kidney disease (CKD). Very young children with CKD frequently have poor spontaneous nutritional intake. Because growth depends strongly on nutrition during early childhood, growth in very young children with CKD is often suboptimal. In this review we will consider the mechanisms and manifestations of inadequate nutritional status in very young children with CKD, mechanisms mediating inadequate nutritional intake, and the optimal nutritional management of this special population. In addition, we suggest an approach to the assessment of nutritional status, including the use of body mass index in infants. Five major nutritional components are considered: energy, macronutrients, fluids and electrolytes, micronutrients, and calcium/phosphorus/vitamin D. The use of adjunctive therapies, including appetite stimulants, treatment of gastroesophageal reflux and gastric dysmotility, enhanced dialytic clearance, and growth hormone, is also briefly discussed.