Abstract: We describe the case of a patient with Neurofibromatosis type 1 (NF1) and Secondary Hypertrophic Osteoarthropathy. On this association so far, hypertrophic osteoarthropathy seems to be related to the vascular involvement of Neurofibromatosis wherein some studies reveal release of PDGF (platelet derived growth factor) by the neural tumor.In order to exclude other causes of secondary Hypertrophic osteoarthropathy all possible causes were excluded.On the basis of clinical findings and exclusion of all other causes, and no literature or case reports suggestive of presence of Hypertrophic Osteoarthropathy in Neurofibromatosis type-1, this finding attains animportant clinching and rare clinical entity and also signifies to include one among the neurological causes of secondary Hypertrophic Osteoarthropathy. The possible pathophysiology of Hypertrophic Osteoarthropathy in patient with neurofibromatosis has been discussed.