The object of this report is to share our experience of conservative management of giant aplasia cutis congenita (ACC) of the scalp with the topical application of basic fibroblast growth factor (bFGF). Complete epithelialization of the 9 × 8 cm sized defect was achieved in 33 weeks. Careful conservative management could eliminate the requirement of surgery for giant ACC defects of the scalp with bone defects and should be tried if surgery is thought to be risky or has consecutive morbidity. Topical bFGF application seems to accelerate healing, also providing a better epithelium for later reconstructive treatments and its usage could be standardized in the future.