Müllerian anomalies are a relatively uncommon occurrence with implications for adolescents and adults as they may result in specific gynecologic, fertility, and obstetrical issues. The exact incidence of Müllerian anomalies is difficult to ascertain. However, clinicians should be suspicious for Müllerian anomalies in cases of primary amenorrhea, pelvic pain, repetitive pregnancy loss, and certain adverse obstetrical outcomes. While for many women a good reproductive outcome can be achieved, counseling, and in particular psychologic counseling, may be needed for some women, especially those with lesions that preclude childbearing and affect normal sexual function.