Paraneoplastic neurological syndromes are well known sequelae of some malignancies but they have never been reported in transitional cell carcinoma of the bladder. A paraneoplastic neurological syndrome characterized by visual changes, glossal spasm and dysphagia associated with an invasive high grade transitional cell carcinoma of the bladder is reported. Neurological symptoms resolved after extirpation confirming a paraneoplastic condition. Recurrent disease was associated with recurrent neurological symptoms and resolved after a complete response to combination chemotherapy.