Pourfour du Petit Syndrome (PdPS) is characterized by signs of oculosympathetic hyperactivity caused by irritation in the oculosympathetic pathway. It has the same etiologies as Horner syndrome, and it's due to irritation and excitation of the oculosympathetic pathway. We present the case of a 64-year-old woman with Pourfour du Petit syndrome due to compression of the second-order cervical sympathetic chain neuron from a dominant and prominent right internal jugular vein compensatory for contralateral agenesis. Internal jugular vein agenesis is a rare developmental vascular anomaly and is asymptomatic in the majority of patients with this condition.