Background: Glomus tumors are rare tumors which usually appear in the dermis of the distal extremities in younger patients. They are categorized as benign, malignant, or of uncertain malignant potential. Given rarity of disease with a wide range of benign versus malignant behavior, the differential diagnosis is also wide and therefore may pose a diagnostic dilemma. Case Report: We present a case of glomus tumor of uncertain malignant potential in the left upper extremity of a 22 year old male, which was initially diagnosed as leiomyosarcoma upon initial excision. After re-excision achieving negative margins, a second opinion from an expert pathologist was sought and the tumor was then more accurately classified as glomus tumor of uncertain malignant potential. Close surveillance ensued with no further adjuvant treatment. Conclusion: This case highlights the difficulties in identifying and classifying glomus tumors which may significantly alter treatment plans, and therefore is considered worthy of presentation.