A 7-month-old white female with Pentalogy of Cantrell was imaged using 64 slice multidetector computed tomography (MDCT) with 3D mapping to better determine the extent of cardiac, thoracic, and abdominal malformations. Complimentary to ultrasound, the use of 3D 64 slice MDCT can facilitate effective diagnosis and treatment planning in cases of Pentalogy of Cantrell.