Type I and II sialidosis are autosomal recessively inherited glycoprotein s
torage disorders. Until now, there has been no published reports of patient
s with these conditions requiring anesthesia. We present the case of a 31-y
ear-old male afflicted with type I sialidosis who underwent a surgical jeju
nostomy. Regional (spinal) anesthesia was carried out uneventfully. We disc
uss the anesthetic challenges posed by patients with type I and II sialidos
is. Airway assessment and management is particularly crucial.