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Pseudomyxoma peritonei is a rare complication of mucinous tumours of appendiceal or ovarian origin. Other associations are malignancies of the colon, urachus and biliary tree. Large amounts of extracellular and peritoneal mucin result in distortion and loss of function of visceral organs. Currently, radiology plays a critical role in diagnosing this rare entity, in follow-up and in predicting the outcome of cytoreductive surgeries.