BACKGROUND Strangulation ileus is caused by external obstruction to the small bowel, which results in ischemia and loss of bowel peristalsis. Low-grade appendiceal mucinous neoplasm (LAMN) is a low-grade adenocarcinoma that arises in the appendix. LAMN is usually asymptomatic but can present with appendiceal rupture and pseudomyxoma peritonei (PMP). This report is of a rare presentation of LAMN with strangulation ileus in a 92-year-old man. CASE REPORT A 92-year-old man was admitted to the emergency room with sudden onset of lower abdominal pain and abdominal distension. Laboratory investigations showed a leukocytosis with a white blood cell (WBC) count of 14.6×10³/μL with 85.5% neutrophils, blood urea nitrogen (BUN) of 26.6 mg/dL, and serum creatinine of 2.6 mg/dL, consistent with acute renal failure. Arterial blood gas analysis showed lactic acidosis (pH of 7.11) with a base excess of -20.8 mmol/L and lactate of 13.7 mmol/L. Abdominal computed tomography (CT) showed ascites and a dilated obstructed closed loop of the distal ileum associated with an external mass (3.9×2.8 cm). An initial diagnosis was of strangulation ileus due to Meckel's diverticulum. Emergency ileocecal resection was performed. Histopathology showed a low-grade mucinous tumor arising from the mucosa of the appendix, consistent with LAMN. At a 13-month follow-up, the patient was well with no tumor recurrence. CONCLUSIONS This report is of a rare case of LAMN that presented as a surgical emergency with strangulation ileus.