Autosomal dominant polycystic kidney disease may present to the emergency department (ED) with vomiting, abdominal pain or hernias, renal insufficiency or failure, or bleeding from cerebral aneurysms. A 37-year-old man presented to the ED with signs and symptoms of incarcerated inguinal hernia. Laboratory studies showed renal failure with anion gap acidosis, and bedside ultrasound showed multicystic kidneys. Computed tomography confirmed the diagnosis. Emergency physicians should be aware of this common connective tissue defect and its serious associated conditions.