Monilethrix is a rare hereditary disorder of hair first described by Walter G. Smith in 1879, and usually transmitted as autosomal dominant trait. It is characterized hy bead like enlargement af the affected hair, alapecia resulting from ha.ir breakage and keratosis pila.l is. This 3 year old female child patient has been suffered from easy destrurtion of beaded scalp hair and follicular hyperkeratosis of the occipital region since infancy. Past hiatory was ncecontributory and the pe,digree of family showed autosomal dominant trait. Physical examinatior was not rernarkable except skin lesian and laboratory findings were within normal limit. Microscopic finding of the affected hair showed alternating constrictians and node. The scalp bivpsy findings showed mild hyperkeratosis, keratotic plug, mild cellular infiltration of papillary derrnia and perifollicular area.