Lipoma of the diaphragm is an extremely rare entity. A case of this disease is herein reported, together with the review of the literature. The patient described was a 70-year-old male, who was admitted to our hospital with an abnormal shadow on his chest X-ray film. The primary tumor, located above the left hemidiaphragm, was successfully resected by video-assisted thoracic surgery, and the final diagnosis of lipoma originating in the diaphragm was made.