Background: Neurothekeomas are rare benign neoplasms typically occurring in young female patients. The usual presentations are small nodules in different anatomical sites commonly involving the face and the upper limb. Case Report: Here we describe a 52 year-old male who had a swelling in the left thumb for 2 years. Histology showed lobulated mass within the dermis containing spindle cells and abundant myxoid matrix. Immunohistochemistry was positive for S100. These findings were compatible with myxoid neurothekeoma. The lesion was completely excised and at the 6-month follow-up the patient was asymptomatic and had no recurrences. Conclusion: This case of neurothekeoma is an exception as the presentation involves thumb involvement which opined as myxoid neurothekeoma and no recurrence of tumor was reported on follow-up.