Renal tubular dysgenesis is a rare disease of fetal kidney differentiation, which became a diagnostic entity with less than 30 years of history and with many etiologic-pathogenic variants. Most reported cases were autosomal recessive, but acquired cases, including drug-induced (ACE inhibitor, NSAID) and twin-twin transfusion-induced ones, were also present. Ischemia and reduced renal perfusion seem to be the common denominator in all cases. We report a new case and for the first time linked this entity to diabetic embryopathy. The patient was 5-hour-old premature girl born at 35 weeks of gestation via emergency cesarean section owing to an ultrasound finding of multiple fetal anomalies, oligohydramnios, and, later, fetal distress. The mother was a 22-year-old G1P1 with diet-controlled gestational diabetes but without other significant past or family medical history. External examination noted multiple dysmorphic features and abnormalities: large anterior fontanelle with open sutures; Potter face; prominent epicanthal folds; hypoplastic nasal bridge and small, low set ears; nuchal edema; bilateral clubfeet with overlapping toes; and sacral dimple. Dissection revealed bilateral renomegaly with mild hydronephrosis and features characteristic of renal tubular dysgenesis. The kidneys showed disorganized glomerulogenesis at various stages with no clear demarcation of the cortical vs medullary zones, and no cysts were identified. Most prominently were short and poorly developed proximal convoluted tubules. All tubules were monomorphic, lined by small darkly stained epithelial cells, positive for EMA, and lacking PAS-positive brush border in the apical surfaces. The other key findings included hepatomegaly, bilateral hypoplastic lungs, and hypoplastic left heart syndrome. The accompanying placenta showed placentomegaly, meconium staining, umbilical cord edema, and fetal thrombotic vasculopathy, reflecting gestational diabetes. Cytogenetic studies (FISH, karyotyping, and signature chip microarray) all showed a normal female 46,XX karyotype. We concluded that renal tubular dysgenesis can be part of a diabetic embryopathy spectrum.