Endoscopic ultrasonography-guided biliary drainage (EUS-BD) is increasingly being used as a drainage technique in cases where endoscopic retrograde cholangiopancreatography (ERCP) cannot be performed. However, in patients with a massive esophageal hiatal hernia, this poses a risk of mediastinitis due to thoracic puncture. Upside-down stomach, first described in 1926 as type IV diaphragmatic hiatal hernia, is a rare condition in which a large portion of the stomach migrates into the thoracic cavity due to organoaxial rotation [1]. There have been reports of ERCP in patients with upside-down stomach [2] [3] but none of EUS-BD. This is the first report of EUS-BD for distal bile duct obstruction in a patient with pancreatic head cancer and upside-down stomach. An 84-year-old woman presented to our hospital with the chief complaint of weight loss. Computed tomography revealed a 35-mm tumor in the pancreatic head, and obstruction of the distal bile duct and duodenum by the tumor was suspected ([Fig. 1]). The patient also had an esophageal hiatal hernia of the upside-down stomach type, and almost all of her stomach had prolapsed into the thoracic cavity ([Fig. 2], [Fig. 3]). We decided to attempt endoscopic ultrasonography-guided choledochoduodenostomy (EUS-CDS) along with duodenal stent placement in the papillary region. A forward-viewing scope was advanced to the duodenum, and a stiff-type guidewire was advanced to the jejunum ([Fig. 4]). The guidewire was left in place, and the endoscope was removed while the loop for the upside-down stomach was released. A convex endoscope (UCT-260; Olympus, Tokyo, Japan) was then advanced over the wire to the esophagogastric junction. The contrast catheter was advanced, and the endoscope was successfully maneuvered around it to the duodenum. Thereafter, EUS-CDS was performed, and a covered self-expanding metal stent (ZEO STENT, 8 × 60 mm; Zeon Medical Inc., Tokyo, Japan) was placed successfully ([Video 1], [Fig. 5]). This case demonstrates that EUS-BD can be safely performed for malignant distal bile duct obstruction with upside-down stomach if EUS-CDS is used. Endoscopy_UCTN_Code_TTT_1AS_2AH Article published online: 17 May 2024 © 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/). Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany