Hypertrophic osteoarthropathy is characterized by clubbing of the digital tips and periosteal reaction of long bones. Most of the cases are associated with malignancy or other conditions such as congenital heart disease, liver cirrhosis, pulmonary fibrosis, biliary atresia, and gastrointestinal polyps. Hypertrophic osteoarthropathy associated with malignancy is rare in children. A few cases of hypertrophic osteoarthropathy in children with nasopharyngeal carcinoma have been reported, however, there has been no report of such case in Korea. We present a case of hypertrophic osteoarthropathy associated with nasopharyngeal carcinoma with lung metastasis in a 14-yr-old boy. In this case, hypertrophic osteoarthropathy regressed after intensive chemotherapy, but subsequently the patient died of progressive lung metastasis.