Woolly hair is a disorder characterized by more or less tightly coiled, usually poorly pigmented, sparse hair occurring over the entire scalp or over the part of it.
A 24 year-old male patient admitted to our outpatient department complaining increased shedding and thinning of the hair, which he suffered since he was 4 years old. His medical history was not remarkable. In his family history, there was nobody in his family affected by the disease. On physical examination, all the hairs over the scalp were sparse, short, thin and curly. A light microscopic examination of his easily plucked hairs revealed a dystrophic root of the hair without root sheath components. The skin and appendages were otherwise normal. No abnormalities were detected in other systems despite detailed examination and investigations. We report this sporadic case of woolly hair because of its rarity and we also reviewed the literature.