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To evaluate outcomes of congenital solitary functioning kidney (SFK) in early childhood.A retrospective study of 32 children diagnosed in utero with SFK owing to unilateral renal agenesis or multicystic dysplastic kidney and followed for 1 to 11.5 years.SFK length was in the compensatory hypertrophy range in 45% of fetal sonographic evaluations from mid-pregnancy, and in 85% on postnatal follow-up. Glomerular filtration rate was below normal range in 44.4%, 12.5% and 0% at <1 year, age 1 to 3 years and thereafter, respectively. Hyperfiltration was detected in 18.5% and 82.6% at <1 year and >3 years, respectively. Hypertension was documented in 35% at age 1 to 3 years but in none at an older age. Proteinuria was absent in all children.Congenital SFK is apparently associated with little or no renal damage in infancy or childhood. Compensatory enlargement of the functioning kidney begins in utero and might serve as a prognostic indicator for normal renal function after birth.