Purpose: Chronic pain is a prominent feature of autosomal dominant polycystic kidney disease (ADPKD) that is difficult to treat and manage, often resulting in a decrease in quality of life. Understanding the underlying anatomy of renal innervation and different etiologies of pain that occur in ADPKD can help to guide proper treatments to manage pain. Reviewing the previously studied treatments for pain in ADPKD can help to characterize treatment in a stepwise fashion. Materials and Methods: We performed a literature search of the etiology and management of pain in ADPKD and the anatomy of renal innervation using Pubmed® and Embase® from January 1985 to April 2014 with limitations to human studies and English language. Results: Pain occurs in the majority of patients with ADPKD due to renal, hepatic, and mechanical origins. Patients may experience different types of pain, which can make it difficult to clinically confirm its etiology. An anatomical and histological evaluation of the complex renal innervation helps with an understanding of the mechanisms that can lead to renal pain. Understanding the complex nature of renal innervation is essential for surgeons to perform a renal denervation procedure. The management of pain in ADPKD should be approached in a stepwise fashion. Acute causes of renal pain must first be ruled out due to the high incidence in ADPKD. For chronic pain, non-opioid analgesics and conservative interventions can be first used before opioid analgesics are considered. If pain continues, there are surgical interventions that can target pain produced by renal or hepatic cysts. Surgical options include renal cyst decortication, renal denervation, and nephrectomy. Conclusion: Chronic pain in patients with ADPKD is often refractory to conservative, medical, and other non-invasive treatments. There are effective surgical procedures that can be implemented when more conservative treatments fail. Laparoscopic cyst decortication has been well studied and results in relief of chronic renal pain in the majority of patients. In addition, renal denervation has successfully been utilized and could be performed concurrently with cyst decortication. Nephrectomy should be reserved for patients with intractable pain and renal failure when other modalities have failed.