Cornelia de Lange syndrome is a multisystem developmental disorder with features of delayed developmental milestones and psychomotor retardation. This syndrome also comprises of facial dysmorphism, hypertrichosis, upper limb deformities, cardiac and gastrointestinal abnormalities. We report a case of hypertrichosis with features of facial dysmorphism and upper limb deformities suggesting a diagnosis of Cornelia de Lange syndrome¹.