Introduction: Racemose hemangioma of the bronchial artery is a rare disease. The diagnosis of racemose hemangioma is difficult when affected patients are asymptomatic, and causes unexplained hemoptysis. Selective bronchial artery embolization has become the first-line treatment for this rare disease. However, feasibility and safety of bronchial artery embolization was not reported in the case with permanent tracheostomy. Case presentation: A 44-year-old woman underwent endoscopic screening during which a circular tumor was detected in the cervical esophagus, which was confirmed as squamous cell carcinoma on biopsy. She underwent cervical esophagectomy with pharyngolaryngectomy through a cervical incision. Reconstruction was performed by free jejunal transfer and permanent tracheostomy. Six weeks after surgery, hemoptysis from permanent tracheostomy was observed. Computed tomography (CT) scan revealed no bleeding around the tracheostomy, and bronchoscopy could not identify the origin of the bleeding due to a large amount of blood clots obscuring the visual field. Bronchial arteriography showed a bent, meandering and dilated bronchial artery, extending to the right bronchus with vascular hyperplasia. We diagnosed primary racemose hemangioma of the bronchial artery, which was successfully treated by transcatheter arterial embolization of the bronchial artery. After embolization, bronchoscopy revealed no bleeding. Conclusion: Findings from this case suggest that bronchial angiography is essential for diagnosis of this disease, and selective bronchial artery embolization appears to be effective in treating this disease in patients with permanent tracheostomy. Accumulation of similar cases will help to elucidate the optimal diagnosis and treatment strategy for this condition.