Chondroid syringoma (CS) is an uncommon, benign, sweat-gland tumor. The usual presentation is of an asymptomatic, slowly growing mass, typically located on the head and neck region. We present a case of a CS located in the right arm of a 43-year-old woman. This tumor developed slowly over 8 years. The diagnosis of CS was made by histopathological examination; it showed an abundant chondroid stroma intermingled with a fibro-adipoid tissue. There was no recurrence after total excision of the tumor with a 2-year followup. The unusual localization of chondroid syringoma made an accurate preoperative diagnosis difficult and diagnosis was achieved only by microscopy. Surgical tumor excision remains the best therapeutic option to avoid relapses of this tumor. Close followup is recommended because malignant transformation, although rare, is possible.