A 66 years old male presented with sudden onset of left-sided weakness, transient slurred speech, and deconjugate gaze. His past medical history is significant for hypertension, dyslipidemia, and genetically confirmed HOS initially diagnosed at the age of 12 and showed TBX5 mutation. At the time of his diagnosis, he had presented with an ASD that reportedly closed spontaneously and pectus carinatum. However, he had no major limb deformity.