A very rare case of a newborn with hypoplastic left heart syndrome and situs inversus totalis is herein reported. Successful surgical treatment of this cardiac malformation was accomplished at our institution and consisted of: modified Norwood stage I procedure, with direct anastomosis of the transected main pulmonary artery to the aortic arch, without any prosthetic material interposition and right ventricle to pulmonary artery polytetrafluoroethylene shunt.