Vitamin D-dependent rickets Type II is a rare autosomal recessive disorder. It usually presents with rachitic changes not responsive to Vitamin D treatment with elevated circulating levels of 1,25-Dihydroxyvitamin D3, thus differentiating it from Vitamin D-dependent rickets Type I. Alopecia of the scalp or the body is seen in some families with Vitamin D-dependent rickets Type II. This is usually associated with more severe resistance to Vitamin D. We report two Saudi brothers with this disease, and review the salient features of this disease with emphasis on the associated alopecia.