We encountered a hilar bile duct cancer patient with intestinal Behçet's disease who manifested various symptoms during a short period in the acute stage after liver resection. Under a diagnosis of hilar bile duct cancer, a 52-year-old Japanese male was referred and admitted to our hospital. Hepatectomy and resection of the extrahepatic bile duct were performed. During the postoperative course, a large number of painful aphthous ulcers developed in the oral mucosa, along with a genital ulcer. A deep excavation-type ulcer was confirmed by colonoscopy in the ileum on the orifice side. We administered colchicine and mesalazine. Thereafter, the patient was treated at the outpatient clinic. Recurrent localized hilar bile duct cancer developed, and the patient died about 8 months after surgery. We present the clinical details of this rare case of hilar bile duct cancer with intestinal Behçet's disease.