Uncombable hair syndrome, also named "pili trianguli et canaliculi" or "cheveux incoiffables", is a rare structural anomaly of the hair shaft first reported in 1973. Both inherited and sporadic forms have been described, characterized by dry and frizzy scalp hair that is impossible to comb. Diagnosis is suspected clinically and confirmed by scanning electron microscopy. The condition is usually isolated, however, several physical abnormalities can be associated. We report the case of a 2(1/2) year old-girl presenting isolated uncombable hair syndrome suspected clinically and confirmed by scanning electron microscopy.