INTRODUCTION AND IMPORTANCE: Volvulus is the twisting of the mesentery of the bowel along its axis. Ileal volvulus is a rare cause of small bowel obstruction. Ileal volvulus coexisting with autosomal dominant polycystic kidney disease has not been reported in the literature previously. CASE PRESENTATION: 65-year male with known history of autosomal dominant polycystic kidney disease (ADPKD) presented with pain abdomen for 5 days, obstipation for 3 days, and multiple episodes of bilious vomiting in the emergency department. Being a suspect of bowel obstruction, X-ray abdomen was done which showed features of small intestine obstruction. Further, to find the etiology of obstruction, contrast enhanced computed tomography (CECT) abdomen was done which showed swirling of the ileal loop and the ileal mesenteric vessels along with transition point in the ileal loop suggestive of ileal volvulus. Exploratory laparotomy with detorsion of the volvulus was done for management. CLINICAL DISCUSSION: Small bowel volvulus, more specifically ileal volvulus, is a rare cause of intestinal obstruction. Patients present with the cardinal features of bowel obstruction, i.e., abdominal pain, distension, vomiting, and constipation/obstipation. Our patient had coexisting ADPKD which further aggravated the clinical presentation. Definitive management of the volvulus includes exploratory laparotomy and detorsion along with resection of the bowel if found ischemic. In our case the bowel was healthy so only detorsion was done. CONCLUSION: Early diagnosis and meticulous exploratory laparotomy is utmost for the management of ileal volvulus. Besides, the secondary etiology (ADPKD in our case) should be managed to prevent future recurrences.