Waardenburg syndrome (WS) is a rare genetic disorder with developmental anomalies of tissues derived from the neural crest and characterized by auditory and pigmentary findings.Failure of neural crestderived melanocyte differentiation results in a spectrum of phenotypic presentations that are subdivided into 4 clinical types.We present a case of Waardenburg type 2 and briefly discuss the genetic basis of phenotypic expression of WS.CASE REPORT An 18-year-old woman undergoing evaluation of intercostal neuralgia was incidentally noted to have one blue and one brown eye (figure).Both pupils reacted equally to light and accommodation.The patient's mother reported that at birth both her eyes were blue but after 2 weeks the left eye changed to brown while the right eye remained blue.At birth she had a tuft of white hair on the frontal area.She had profound sensorineural hearing loss since birth and communicated via sign language.No other family member had this condition.Her mother's eyes were brown and the color of her father's eyes was unknown.The patient was subsequently lost to follow-up.The differential diagnosis included oculocutaneous albinism, piebaldism, oculocerebral hypopigmentation syndrome, congenital Horner syndrome, neuroblastoma, and WS.Oculocutaneous albinism is characterized by generalized hypopigmentation of skin, hair, and eyes due to defective synthesis of melanin. 1 Since the