The nephrotic syndrome is a rare, idiosyncratic adverse renal effect of lithium that can occur with therapeutic plasma lithium levels. The syndrome is usually reversed by discontinuation of lithium treatment but may require corticosteroids. Renal biopsies reveal fusion of the foot processes of renal epithelial cells, referred to as "minimal change disease." No particular variable identifies individuals at risk for developing the nephrotic syndrome while taking lithium. The authors review the eight published cases in the English-language literature and present the case of an adolescent who developed lithium-induced nephrotic syndrome.