We herein report a case of a 45-year-old white male who referred to the emergency department for a right pneumothorax. A chest tube was emergently placed. Due to incomplete lung reexpansion, the patient underwent a right thoracoscopy disclosing the presence of several kinky vessels consistent of localized pleural angiomatosis, and a talc pleurodesis was performed. Computed tomographic scan and angiography confirmed an anomalous vascular connection between systemic and pulmonary circulation. Thus, a vascular percutaneous transcatheter embolization of the abnormal vessel was successfully executed and the patient was discharged without consequence.