Polymyositis as a paraneoplastic syndrome associated with hepatocellular carcinoma is quite rare; only a few cases have been reported. We report a case of a 50-year-old female who presented with subacute quadriparesis, neck muscle weakness, elevated creatinine phosphokinase, a myogenic pattern on the EMG and was diagnosed as having polymyositis, a paraneoplastic syndrome associated with hepatocellular carcinoma with negative hepatic viral markers and a positive ANA. Improvement in patient symptoms and areduction in creatinine phosphokinase, which occurred after lobectomy, supports this rare association.