IFT140 is a component of the intraflagellar transport-complex A involved in retrograde ciliary trafficking of proteins into primary cilia. Monoallelic IFT140 variants have been identified as an important cause of adult-onset autosomal dominant polycystic kidney disease (ADPKD), accounting for ∼2% of prevalent cases. Patients with ADPKD-IFT140 usually present in later life with small numbers of large cysts and rarely develop kidney failure. Here, we report 3 genetically resolved cases of ADPKD-IFT140 diagnosed in childhood or infancy from 3 unrelated pedigrees with ages at presentation ranging from in utero to 14 years.