Neurofibromatosis type 1 (NF 1) or Von Recklinghausen's disease is an autos
omal dominant condition characterized by multiple skin cafe-au-lait lesions
and multiple neurofibromas. In the head and neck neurofibromas have a pred
ilection to arise in th deep planes of the neck. Surgical management is nea
rly always required for functional or cosmetic reasons or to exclude the po
ssibility of malignant transformation. We present four cases of neurofibrom
a of the head and neck and illustrate the difficulties inherent in managing
this condition, particularly the age range in which it may occur, the risk
of malignant transformation, the possibility of abnormal bleeding and the
morbidity that may be associated with the surgical resection. The role of m
agnetic resonance (MR) scan in the assessment and monitoring of these lesio
ns is highlighted. We also report a unique association of a pharyngeal pouc
h with NF 1 in a young (25-year-old) patient.