Polycystic kidney disease in a common inherited disorder accounting for 8-10% of cases of end-stage renal disease. The enlarged kidneys often produce pain and hematuria but rarely obstruction of surrounding organs. We report a case of autosomal dominant polycystic kidney disease producing symptomatic duodenal obstruction and malnutrition. Duodenal obstruction should be considered in the differential diagnosis of a patient with polycystic kidney disease and intermittent or persistent nausea and vomiting.