Introduction:
Situs inversus is a rare congenital anomaly with an incidence of about 1 in 15000 of the general population (1). When associated with dextrocardia, it is known as situs inversus totalis (SIT) in which we find complete inversion (mirror-image) of the position of the thoracic and abdominal viscera. This disorder in the position of the heart chambers may be isolated or associated with other cardiac defects (2). The incidence of associated cardiac defects in patients with SIT is nearly 4%, commonly with transposition of the great vessels (3, 4). Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly characterized by failure of normal connection of one or more of (but not all) the pulmonary veins to the left atrium. Alternatively, the anomalous pulmonary veins connect to the right atrium or one of its tributaries (5, 6).  The overall prevalence of PAPVC among general population has been reported to be between 0.4–0.7% (7). Its association with SIT is very rare. Herein, we report a rare case of SIT associated with PAPVC and secundum atrial septal defect (ASD) in a 30-month-old girl who underwent successful surgical repair.