Glomus tumors are uncommon benign tumors developing from the neuro-myo-arterial glomus body. They are typically located in the fingers. The extra-digital involvement is unusual and makes diagnosis difficult. Only few cases have been reported in literature. We report an exceptional case of a glomus tumor of the lower leg in a 65-year-old male. The diagnosis was clinically suspected and confirmed by a biopsy. Surgical excision gave immediate pain relief. The aim of this report is to make the surgical community more aware of this entity based on the analysis of our own experience and a review of the literature.