The infantile intestinal obstruction associated with situs inversus totalis and polysplenia is extremely rare, with only a few cases reported in the literature. Furthermore, the management of this association is complicated. We report a case of a 2-month-old boy with intestinal obstruction due to malrotation and volvulus with thin translucent omentum sac encasing the small intestine associated with situs inversus totalis, polysplenia, and pulmonary hypertension. To the best of our knowledge, this is the first case of situs inversus totalis with polysplenia, pulmonary hypertension, and intestinal obstruction due to malrotation and volvulus with thin translucent omentum sac encasing the small intestine.