Von Recklinghausen’s disease is an autosomal-dominant hereditary disorder that is rarely accompanied by rhabdomyosarcoma of the head and neck in an adult. We report a case of von Recklinghausen’s disease complicated by rhabdomyosarcoma of the tongue and thyroid cancer. A 38-year-old man was referred to our hospital because of spontaneous pain of the left side of the tongue. An intraoral examination revealed a palpable hard tumor measuring 24 × 17 mm. Because we suspected a benign tumor, we performed a partial glossectomy with the patient under local anesthesia. Histological examination showed an embryonal rhabdomyosarcoma. We performed an additional resection with general anesthesia. Three weeks later, the patient underwent a thyroid resection and neck dissection. The histopathological examination revealed a papillary carcinoma. The patient declined to receive adjuvant chemotherapy as well as radiotherapy. He has not had recurrence or metastasis in the 4 years since the resection.