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Horseshoe kidney is the most common renal fusion anomaly occurring in 0.15–0.25% of the general population. Horseshoe kidney is usually asymptomatic but may present with disease-like infections, urolithiasis, malignancy, polycystic disease, and other associated anomalies that may require intervention. Polycystic horseshoe kidney is rare, its association with uterine prolapse has not been reported in the literature, and it can only be postulated as an associated risk factor for uterine prolapse when found in the pelvis. The authors reviewed rare associations of horseshoe kidney and the management of these associated anomalies and disease conditions with the clinical vignette of a 60-year-old female with low-lying/lumbo-pelvic polycystic horseshoe kidney and hepatic cyst, and concomitant uterine prolapse requiring total abdominal hysterectomy at a Senegalese Hospital.