Waardenburg's syndrome is characterized by a broad nasal root, pigmentation disturbance and congenital deafness while aganglionosis is described as the partial or complete lack of ganglion cells in the alimentary tract. This report describes a five-day-old male infant with Waardenburg's syndrome associated with total aganglionosis of the colon, ileum and distal jejunum and draws attention to the causal relationship between these two entities.