We present a case of reconstruction of severe aplasia cutis congenita. Initial treatment with silver sulphadiazine dressings failed and local scalp repair of the defect at age 3 months also failed to provide a stable reconstruction. At age 9 months the infant was experiencing increasing apparent hydrocephalus, shunt infection and brain herniation. The definitive reconstruction was performed with a split rib cranioplasty and a free latissimus dorsi muscle flap. The reconstruction was successful and the infant has a normal head shape and no neurological deficit 12 months postoperatively.