Although the clinical features of syringoma are usually scattered papules, especially around the eyes, there are several forms showing atypical presentation and location: unilateral, eruptive, acral, and linear. The uncommon characteristic features (linear and unilateral) may confuse and delay the differential diagnoses of syringoma. We report a case of a 35-year-old female who presented with a 10-year history of linear, skin-colored papules on her right arm. Histopathologic findings were consistent with typical syringoma, showing comma-like tubal structures lined by eccrine glandular cells. This case suggests that syringomas need to be included in the differential diagnosis of unilateral, linear, papular or plaque skin lesions.