Background: Polycythemia vera has been found in a wide range of hematopoietic tissue disorders, but rarely concomitant with renal abnormality. To our knowledge there is only one available report of a patient with polycythemia vera concomitant with renal angiomyolipoma, and the report does not cover the clinical course of this rare finding. Objectives: To report the presentation and clinical course of a patient with polycythemia vera concomitant with right renal angiomyolipoma. Methods: We retrospectively reviewed the medical records of a patient with polycythemia vera who presented with renal angiomyolipoma. Results: Our patient presented with a right renal mass and polycythemia vera diagnosed by a point mutation in the gene for Janus kinase 2. The mass was diagnosed as renal angiomyolipoma on histopathology. After partial nephrectomy, she was prescribed a low dosage of hydroxyurea without the return of erythrocytosis for a year. Conclusions: Polycythemia vera responded well to low dosage hydroxyurea, which controlled erythrocytosis after removing the renal mass.