Polysplenia syndrome (PS) is rarely encountered in elderly. It is characterized by multiple spleens associated with various cardiac and gastrointestinal abnormalities including partial or complete agenesis of dorsal pancreas. Situs inversus totalis (SIT) is a rare congenital anomaly with mirror image of viscera combined with dextrocardia. Occurrence of SIT and PS in the same patient is exceedingly rare. We present the first case of adult PS with SIT accompanied with annular pancreas.
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