Cholelithiasis is being diagnosed with increased frequency in the very young. We encountered 12 patients over a 5-year period in whom cholelithiasis was diagnosed prior to age 1 year. The earliest diagnosis was made in utero during the seventh gestational month. While most of the children suffered from complex medical disorders, 4 of the 12 had no recognizable predisposing factors. Eight of the 12 were treated at some point prior to diagnosis with central parenteral nutrition. Three presented with infectious complications requiring prompt operative intervention. Two children underwent elective cholecystectomy nearly 2 years after the diagnosis for symptoms of biliary colic. Of the remaining seven, spontaneous stone resolution was documented in three, while four remain asymptomatic after a minimum of years follow-up. The spectrum of presentations in this series highlights the uncertainties regarding the etiology, natural history, and treatment of this increasingly more common disorder.