Cystic fibrosis and the various conditions associated with ciliary dysmotility are well known to the otolaryngologist. Although the underlying pathophysiology and clinical courses of these diseases are quite different, they both involve chronic and acute infection in the paranasal sinuses. Some patients may suffer from persistent ear disease beyond early childhood. A high index of suspicion and early diagnosis are key to managing these conditions. The otolaryngologist often is the first to suspect and confirm the diagnosis and may have an important role in the overall management of these patients.