Camptodactyly is a pediatric hand condition, the treatment of which remains controversial. The authors' aim was to improve patient care through clarifying the definition of camptodactyly and indications for surgical and/or conservative management, summarizing outcomes, and defining risks. A systematic review was conducted of articles in all languages on outcomes following surgical and/or conservative management of idiopathic camptodactyly in children using MEDLINE (Medical Literature Analysis and Retrieval System Online), PubMed, EMBASE (Excerpta Medica database), AMED (Allied and Complementary Medicine), and CINAHL (Cumulative Index of Nursing and Allied Health Literature) (until January 2017). The primary outcome was posttreatment flexion contracture, and the secondary outcomes were indications for surgery, complications, and patient satisfaction. Database searching generated 16 final articles, with 7 case series and 9 retrospective cohort studies. There was a lack of consistency on the definition of camptodactyly and in outcome reporting. All 16 studies received a "Weak" global rating and demonstrated low-quality evidence, suggesting that treatment of camptodactyly with operative or nonoperative measures reduces the degree of flexion contracture in most patients (from pretreatment averages of 20°-85° to posttreatment averages of 5°-37°). There was general agreement that surgery should be reserved for contracture >30° or failure to respond to conservative management. Surgery generally led to more complications compared with conservative management. Only one study reported on functional limitations, and another reported on patient-reported outcomes. Current evidence of the effectiveness of camptodactyly treatment in addressing both joint-specific deformity and patient-perceived function and appearance is insufficient to guide patient care. Future research may consider the development of decision aids to guide patients and families through selecting management strategies and to promote shared decision making.