Clinicians may encounter several different lesions of the scalp and skull that present with tissue loss of varying degrees. Parry-Romberg syndrome, bilateral parietal osteodystrophy, Gorham disease, hyperostosis frontalis interna, chronic anemia, and renal osteodystrophy all result in atrophy of tissue layers pertaining to the skull or scalp. Most of these pathologies have an unknown mechanism, and surgical treatment is often reserved for severe symptomatic presentations. Even when asymptomatic, recognition of each disease and the layers that it effects are useful when planning unrelated surgical procedures to avoid neurological morbidity or mortality. We detail the presentation and treatment options for each disease entity below, as well as highlight a case of hyperostosis frontalis interna.