We present monozygotic girls born to consanguineous parents, both harboring a potentially harmful homozygous missense variant in CRB2. Their phenotype aligns with the known features of CRB2-related features, including cystic kidney disease, early-onset nephrosis, ventriculomegaly, gray matter heterotopia, and cardiomyopathy [6–8, 12, 13]. This evidence concerns the gene CRB2 and cardiomyopathy.