Introduction:
Neurofibromatosis is a neurocutaneous syndrome,
mostly familial in origin and believed to arise from a defect
in differentiation of the primitive ectoderm. There are two
distinct forms of neurofibromatosis – type-1 and type-
21. Neurofibromatosis type-1 (NF-1), previously known
as Von Recklinghausen disease, is an autosomaldominant
disorder with a frequency of approximately 1
in 3,000 births2. Neurofibromatosis type-1 classically
presents with hyperpigmentation, known as café-au-lait
spots, and multiple neurofibromas3. Plexiform
neurofibroma is considered as an uncommon skin tumor.
The involvement of the lower limb is rare. The management
of patients with plexiform neurofibroma is not well defined
and aims mostly at controlling symptoms4. In this report,
we present a rare case of neurofibromatosis with right
lower limb gigantism.