Anti-neutrophil cytoplasmic antibodies associated vasculitis (AAV) are small vessel vasculitides that can present with a spectrum of manifestations depending on the severity of organ involvement. Pseudotumor cerebri is a disorder characterized by elevated cerebrospinal fluid pressure, without any evidence of intracranial pathology. The association between pseudotumor cerebri and chronic renal failure is uncommon and data are limited. We report a case of a young obese female who presented clinical manifestations of pseudotumor cerebri and chronic kidney disease, whose etiological study showed elements of AAV. To our knowledge, this association has not been previously reported.