Granulomatosis with Polyangiitis (GPA) can present with Cerebral Venous Sinus Thrombosis (CVST), Chronic Suppurative Otitis Media, and Lower Motor Neuron (LMN) Facial Palsy. However, an association between CVST and Hyperhomocysteinemia in GPA has not previously been reported. Here, we report a case of CVST and Hyperhomocysteinemia in Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) positive GPA without renal involvement.