Localized agenesis of the scalp is a well-recognized phenomenon, with over 300 cases reported in the literature. These defects have previously been called congenital ulcer of the newborn, Streeters spots or aplasia cutis congenita (ACC). First described 160 years ago, ACC is recognized as a heterogenous group of disorders all having focal absence of scalp. Type III ACC, Adams-Oliver syndrome, consists of a scalp defect associated with a distal limb anomaly. Typically, the lesions appear as small ulcerations which may heal spontaneously. Larger lesions associated with underlying bony defects occur, and may cause death secondary to infection or hemorrhage from the sagittal sinus. We report a case of Adams-Oliver syndrome that required surgical excision and closure because of involvement of the sagittal sinus documented by multiple imaging modalities.