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This article will describe the different variants of the unicornuate uterus, their clinical presentation and imaging findings, as well their associated complications. We will also review the associated renal anomalies. Patients' symptoms and their imaging findings will vary depending on the unicornuate subtype. Radiologic evaluation includes a combination of hysterosalpingography, sonography, and magnetic resonance imaging. Complications include obstetric ones related to the small uterine size and endometriosis and ectopic pregnancies when a cavitary rudimentary uterine horn is present. Radiologists should be familiar with all variants of the unicornuate uterus as well as their clinical presentation and associated imaging findings.