Uterine adenosarcoma is a rare biphasic tumour comprising benign epithelial and malignant mesenchymal components. It predominantly affects women in their 60s–70s but can occur at any age. The tumour usually originates in the endometrium and presents as a polypoid mass causing abnormal uterine bleeding, pain, or pelvic pressure. Risk factors include prior pelvic radiation, tamoxifen use, and endometriosis. Histologically, adenosarcomas are low-grade but can have aggressive high-grade variants with sarcomatous overgrowth, which worsens prognosis. Molecular studies reveal alterations in pathways such as PIK3CA/AKT/PTEN amplification, providing potential therapeutic targets. Diagnosis relies on imaging (ultrasound, MRI) and histopathological evaluation by gynaecologic pathologists. Surgical staging is essential, and total hysterectomy is the standard treatment. Adjuvant therapies, including chemotherapy and hormonal therapy, are considered for advanced disease. Recurrence and metastases are challenging, with limited survival improvements. Recent advances in molecular profiling and emerging therapies, including immune checkpoint inhibitors, offer hope for better management strategies. Multidisciplinary care and collaborative research are critical to improving outcomes for this rare malignancy.