Hypertrophy of Pacinian corpuscles is a condition that has been reported very infrequently. It was thought not to be connected with neurofibromatosis. A 24-year-old female with a lifelong history of neurofibromatosis type 1, who presented with left ring finger pain and decreased sensation for over 1 year is reported. Histopathology showed hypertrophy of Pacinian corpuscles. Hypertrophy of Pacinian corpuscles should be considered in the differential diagnosis of any patient with persistent pain localized to the distal palm or digits. This is the first case reported in a patient with neurofibromatosis. The finding of this lesion in a patient with neurofibromatosis is somewhat intriguing, though the relationship of these two entities is unclear.