Paraneoplastic neurological syndromes are well-known sequelae of some cancers. Based on the current literature, this is the first report of a complete ophthalmoplegia preceding the diagnosis of a myxoid liposarcoma associated with the anti-Hu antibody. An 83-year-old woman presented with a several-month history of progressive ophthalmoplegia without any other neurological symptoms or signs. After resection of her myxoid liposarcoma grade 1, her ophthalmoplegia resolved. Her paraneoplastic syndrome was associated with the anti-Hu antibody. It is important to consider an underlying malignancy when an isolated, complete ophthalmoplegia presents subacutely and when other more common aetiologies are ruled out.