First branchial cleft sinuses account for less than 1% of all branchial cleft anomalies. A thorough understanding of the embryology and developmental anatomy is essential for successful management. Nine cases of first branchial cleft sinuses treated at The Hospital for Sick Children by the Department of Otolaryngology between 1984 and 1990 were reviewed. A large proportion of these lesions were initially misdiagnosed despite significant symptomatology. This resulted in a high rate of infectious complications. Early diagnosis, prompt control of infection, and early surgical excision are recommended. Current methods of diagnosis and treatment are discussed along with techniques for facial nerve preservation.