Purpose: This study aims to highlight the characteristics and prognosis of undifferentiated carcinoma with rhabdoid features, a rare malignancy within the spectrum of malignant rhabdoid tumours (MRT), through a case report and review of current literature. This is tumour is infrequently encountered in the clinical setting and presents a therapeutic challenge due to its aggressive nature and limited understanding. Case Presentation: A 51-year-old male presented to his primary care doctor with intermittent abdominal pain, leading to a diagnostic computed tomography (CT) revealing an abdominal mass presumed to be a jejunal gastrointestinal stromal tumour (GIST). Subsequent laparoscopy revealed a large, perforated tumour in the abdomen containing blood, prompting an en bloc resection. Despite recovering well from surgery and commencing initial chemotherapy, the patient experienced a large bowel obstruction from multiple peritoneal metastasis, necessitating a stoma placement and palliative care input. The patient passed away within 3 months of initial CT diagnosis. Discussion: A comprehensive literature search identified sparse documentation on undifferentiated carcinoma with rhabdoid features, emphasising its rarity and challenge with diagnosis and management. These studies have highlighted its aggressive behaviour, poor response to standard treatments, and grim prognosis. Conclusion: Undifferentiated carcinoma with rhabdoid features presents significant clinical challenges due to rarity, aggressiveness, and limited treatment options. This study offers insights into its presentation, histopathology, and prognosis, underlining the need for further research to enhance diagnostic and therapeutic approaches to improve patient outcomes.