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BACKGROUND: A few small studies have reported increased prevalences of polycystic ovary syndrome (PCOS) and symptoms of androgen excess in women with type 1 diabetes. PURPOSE: We performed a systematic review and meta-analysis of studies evaluating androgen excess symptoms and PCOS in women with type 1 diabetes. DATA SOURCES: The Entrez-PubMed and Scopus electronic databases were used. STUDY SELECTION: We selected studies addressing androgen excess signs, symptoms, and disorders in girls, adolescents, and adult women with type 1 diabetes. DATA EXTRACTION: The main outcome measures were prevalences of PCOS, hyperandrogenemia, hirsutism, menstrual dysfunction, and polycystic ovarian morphology (PCOM). DATA SYNTHESIS: Nine primary studies involving 475 adolescent or adult women with type 1 diabetes were included. The prevalences of PCOS and associated traits in women with type 1 diabetes were 24% (95% CI 15-34) for PCOS, 25% (95% CI 17-33) for hyperandrogenemia, 25% (95% CI 16-36) for hirsutism, 24% (95% CI 17-32) for menstrual dysfunction, and 33% (95% CI 24-44) for PCOM. These figures are considerably higher than those reported earlier in the general population without diabetes. LIMITATIONS: The data collected in the original studies were heterogeneous in age, race, ethnicity, and criteria used for the diagnosis of PCOS; yet, we used a quality-effects model in the meta-analyses to overcome this limitation. CONCLUSIONS: PCOS and its related traits are frequent findings in women with type 1 diabetes. PCOS may contribute to the subfertility of these women by a mechanism that does not directly depend on glycemic/metabolic control among other negative consequences for their health. Hence, screening for PCOS and androgen excess should be included in current guidelines for the management of type 1 diabetes in women.