Abstract Background Heteropagus twinning is a rare birth defect in 1 in million live births. Epigastric heteropagus is the most frequent type, in which a smaller dependent (parasite twin) is attached to the body of the host (autosite twin) to the thorax and umbilicus. Several congenital anomalies have been previously described in the host twin. We describe the first case of epigastric heteropagus twinning with dextrocardia and situs inversus in which a successful surgical separation is performed. Case presentation A 48-h-old female sex twins were presented to Bahawal Victoria Hospital. A careful anatomic study using contrast X-ray, magnetic resonance imaging, and echocardiogram showed epigastric heteropagus twinning and situs inversus in autosite twin. A successful surgical separation was performed, and the parasite twin was removed from the body of the autosite twin. Conclusions Antepartum screening detects congenital malformations; however, several pregnancies are unsupervised in underdeveloped countries. In the case of heteropagus twinning, careful surgical separation in the absence of complications leads to the favorable survival of the autosite twin.
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