Craniofacial surgeons are rarely presented patients with extreme hydrocephalic macrocephaly due to early diagnosis and treatment of the hydrocephalus. Macrocephaly can significantly limit or prohibit mobility, hygiene and can drastically change lifestyle and developmental issues. The authors herein report on four consecutive total cranial vault reduction cranioplasty procedures for correction of hydrocephalic macrocephaly. The patients had a reduction in cranial volume ranging from 111-641 mL. All patients survived the procedure. Improvement in head control and aesthetics were improved in all patients. All of the patients required at least one shunt revision following the procedure. We conclude that total cranial vault reshaping is safe and effective for the treatment of macrocephaly secondary to hydrocephalus.