Acinic cell carcinoma (ACC) is an uncommon malignant tumor of the salivary glands that is difficult to diagnose. It grows slowly and shows distant metastasis rarely. We experienced a case of recurrent ACC in the parotid gland with cardiac metastasis and hypertrophic osteoarthropathy. The 29-year-old man had been suffering from severe multiple bones and joints pain for 2 months. Ten years earlier, he underwent superficial parotidectomy due to a right subauricular mass. The mass was diagnosed with ACC. After surgery, the tumor recurred twice. Then the patient was diagnosed with cardiac metastasis via positron emission tomography-computed tomography and trans-thoracic echocardiography. He also had hypertrophic osteoarthropathy with multiple bone metastasis. He was given palliative radiotherapy and conservative treatment. ACC in the parotid gland with cardiac metastasis and hypertrophic osteoarthropathy has not yet been reported in literature. From this case, it is recommended to evaluate multiple distant metastasis in the ACC of the parotid gland when joint and bone pain are present.