Vascular endothelial growth factor (VEGF) is a cytokine overexpressed in hypoxic and malignant pathologies. VEGF induces vascular hyperplasia, new bone formation, and edema. These histological abnormalities characterize hypertrophic osteoarthropathy. We describe a case of pulmonary hypertrophic osteoarthropathy with high circulating VEGF levels. Removal of the lung tumor led to a dramatic disappearance of the skeletal abnormalities and to reduction of circulating VEGF levels. Histochemical studies of the excised tumor confirmed abnormal VEGF production.