Müllerian duct anomalies include a wide spectrum of genital tract defects resulting from a development alteration of the genital tract. An 11-year-old girl with a hybrid septate variety uterus with left hemi-obstruction was identified. Beside preoperative imaging evaluation done with ultrasound, magnetic resonance, and hysterosalpingography, a combined laparoscopic and hysteroscopic procedure was fundamental for the diagnosis. The surgical procedure also allowed us to perform a successful and safe treatment. We propose that the American Fertility Society classification of these anomalies should be revised and that hybrid entities should be considered in the differential diagnosis of genital tract malformations.