Basal ganglia (BG) malformations are a hallmark of most TUBB3-CFEOM and TUBB3-MCD variants as well as many other tubulinopathies, and include varying degrees of abnormal rotation and asymmetrical enlargement of the caudate head and hypoplasia of the caudate body, with accompanying dilatation of the anterior horn of the lateral ventricle, hypoplasia of the anterior limb of the internal capsule with fusion of the caudate head and underlying putamen, and rounded globular-appearing thalami (Figures 2, 3). The gene discussed is TUBB3; the disease is tubulinopathy.