Albright`s hereditary osteodystrophy is an inherited syndrome that encompasses endocrinologic anomaly of pseudohypoparathyroidim of less commonly, pseudo-pseudohypoparathyroidism and various physical stigmata such as mental retardation, short stature, skeletal anomaly of the hands, abnormal dentition, round facies, and osteoma cutis. Primary osteoma cutis in this syndrome presents at birth or in early infancy, preceding most of the other manifestations. This case is a typical presentation with osteoma cutis as the sole initial manifestation. Rather unfamiliar to dermatologists, Albright`s hereditary osteodystrophy still deserves to be included in the differentials when an isolated case of osteoma cutis presents in a young child.