Purpose of Review <p>Müllerian anomalies are recognized as a significant risk factor for the development of endometriosis. The purpose of this review is to synthesize current evidence on the association between Müllerian anomalies and endometriosis and to present our clinical experience with diagnosis and management strategies in these complex cases.</p> Recent Findings <p>Recent meta-analysis data demonstrate that 47% of patients with obstructive müllerian anomalies develop endometriosis, compared to 19% with non-obstructive anomalies and 21% without anomalies. While surgical correction of obstructive anomalies is often associated with symptom improvement, the degree of endometriosis regression postoperatively remains variable. Some studies report complete resolution of symptoms and endometriotic lesions, while others document persistent disease.</p> Summary <p>Müllerian anomalies, particularly obstructive variants, are strongly associated with an increased risk of endometriosis, likely due to the pathophysiological mechanism of retrograde menstruation. Clinical management requires a thorough understanding of the müllerian anomaly type, and further evidence is needed to determine if concurrent endometriosis treatment during corrective surgery is beneficial.</p>