woolly hair nevus is a rare condition , characterized by unruly and tightly coiled hair , which is not genetically determined .
a 5-year - old girl child presented with abnormal patch of hair since 2 years of age .
her parents noticed a single patch of curling and coiling of hair along with altered texture over the left side of scalp .
her parents felt the patch to be unruly and unsightly and hence attempted repeated tonsuring . in spite of this
, we noticed a solitary circumscribed patch of size 64 cm located over the left frontoparietal region of scalp .
the hair over the patch had an altered texture , was lighter in color , thinner , tightly coiled , and curled giving an unkempt appearance [ figure 1 ] .
based on the above findings , we arrived at a diagnosis of localized woolly hair nevus .
a well - circumscribed patch of tightly coiled woolly hair hpe of scalp showing normal hair follicle and appendages
woolly hair is characterized by tightly coiled hair occurring over the entire scalp or part of it in an individual of non - african origin .
it is a nevoid condition characterized by a circumscribed patch of unruly and curled hair with an altered texture .
this condition may be associated with melanocytic or epidermal nevi , mongolian spots elsewhere on the skin .
ocular defects like persistent pupillary membrane , retinal defects , and precocious puberty have also been associated .
woolly hair nevus is not associated with cardiac abnormalities as in carvajal or naxos disease in which desmoplakin and plakoglobin mutations are known to occur , respectively .
our patient presented with an isolated woolly hair nevus without any associations , which has been reported very rarely .
however , this patient must be followed up regularly to detect any cardiac defects which may present later .
 woolly hair nevus is a rare non - hereditary focal condition characterized by unruly and tightly coiled hair . 
 it can appear in childhood or adolescence and may be associated with epidermal or melanocytic nevus . 
 patients presenting with woolly hair must be examined completely to rule out cardiofaciocutaneous and noonan syndrome . 
INTRODUCTION
CASE REPORT
DISCUSSION