Cutaneous leiomyoma is an infrequently occurring benign tumor that arises from smooth muscle in the skin. Pilar leiomyoma, a subtype of cutaneous leiomyoma, arises from the arrector pili muscle associated with hair follicles. Pilar leiomyoma, particularly in the head and neck region, is rare and frequently misdiagnosed. We report one patient who developed pilar leiomyoma of the nasal dorsum, explore the differential diagnosis and review the characteristics of previously reported individuals with pilar leiomyoma of the nasal dorsum.