Congenital atrichia with papular lesions is a rare, autosomal recessive and irreversible form of total alopecia of the body hair characterized by hair loss soon after birth and the development of keratinfilled cysts or horny papules over extensive areas of the body. The condition is associated with a mutation of the human hairless gene on chromosome region 8p12. We report a 1-year-old boy presenting with the absence of scalp and body hair since birth. On examination, he had complete absence of hair on the scalp, eyebrows, and eyelashes. Multiple, discrete, pearly-to-skin-colored papules of 1-3mm in size were present over the scalp. The skin biopsy from a scalp papule revealed normal overlying epidermis with multiple keratin cysts and hypoplastic hair follicles in the upper dermis.