Urinary bladder paraganglioma in a case of von Hippel-Lindau disease

Pheochromocytoma is a well-known association in patients with von Hippel-Lindau disease. However, extra-adrenal pheochromocytoma or paraganglioma with this association is rare. We describe a patient with von Hippel-Lindau disease who presented with haematuria and was diagnosed to have a urinary bladder paraganglioma, which is an extremely rare tumour. Herein we report this case which, to the best of our knowledge, represents the first case highlighting the association of urinary bladder paraganglioma with von Hippel-Lindau disease.