We present a rare case of a synchronous primary lung cancer adjacent to a hamartoma. A 71-year-old woman was admitted with congestive heart failure due to acute myocardial infarction. A chest radiogram on admission showed pulmonary edema with a tumor shadow in the right upper lung field. Because histological diagnosis was not obtained preoperatively, a wedge resection of the lung was conducted using video-assisted thoracoscopic surgery. The histopathological examination confirmed the coexistence of an adenocarcinoma with a chondromatous hamartoma. Right upper lobectomy was performed followed by excision of the mediastinal lymph nodes. Although hamartoma is generally considered to be a benign neoplasm, there have been several reports of increased risk to lung cancer in patients with a chondromatous hamartoma. Therefore, we recommend that patients with a hamartoma should be submitted to a complete evaluation and to regular follow-up, considering the risk to associated synchronous malignancy.