The authors report a case of xanthogranulomatous cholecystitis in a 63-year-old woman hospitalized because of a right hypochondrium mass. Ultrasonography, computed tomography, and celiac arteriogram showed a gallbladder tumour. Surgical management allowed subtotal tumour resection. Diagnosis of xanthogranulomatous cholecystitis was established by histological examination. Twenty-two months later, patient is in good health. From this observation and a world-wide literature review (90 previously reported cases), the authors analyse the clinical, pathological, and pathophysiological features of this very unusual entity.