Polycystic ovary syndrome (PCOS) is the most common endocrine disorder among reproductive-aged women; however, to date there has been no synthesis of the burden of PCOS specifically among indigenous women. We aimed to systematically identify and collate studies reporting prevalence and clinical features of PCOS among indigenous women worldwide. We performed a comprehensive search of six databases (Ovid MEDLINE, MEDLINE In Process & Other Non-Indexed Citations, EMBASE, EBM reviews, CINAHL, and SCOPUS) supplemented by gray literature searches and the screening of reference lists. A narrative synthesis was conducted. Fourteen studies met inclusion criteria; however, one was excluded as it assessed only children and adolescents younger than 15 years, with limited clinical relevance. Studies examined indigenous women from Australia, Sri Lanka, New Zealand, and the United States. Prevalence of PCOS was reported in only four studies and ranged from 3.05% for women in Sri Lanka to 26% for women in Australia. All included studies reported on at least one clinical feature of PCOS. Of the studies that reported on a comparison group from the same country, there was evidence of more severe features in indigenous women from New Zealand and the United States. The limited evidence available warrants further investigation of the burden of PCOS in indigenous women to build the knowledge base for effective and culturally relevant management of this condition.