We report a case of neuromyelitis optica (Devic's syndrome) with hepatocellular carcinoma.

A 70-year-old male with hepatocellular carcinoma presented with bilateral visual loss. A relative afferent pupillary defect was not observed in either eye due to bilateral mydriasis. On brain MRI, there was no specific finding, however, on spine MRI, multiple and severe myelopathies were observed. After high-dose methylprednisolone pulse therapy, the visual acuity was 0.03 in the right eye and counting fingers at 30 cm in the left eye.

We encountered a case of neuromyelitis optica involving bilateral optic neuropathy in hepatocelluar carcinoma. Therefore, in patients with an optic neuropathy of uncertain etiology, clinicians should consider performing a systemic evaluation.