Follicular mucinosis is a rare disorder of unknown etiology characterized by accumulation of mucin in the sebaceous glands and outer root sheaths of the hair follicles. It is divided into a primary benign type and a secondary type mostly associated with lymphomas. No effective standard therapy for follicular mucinosis is available. We describe the case of a 21-year-old Caucasian male who had papules, nodules, and erythematous plaques on his left shoulder, left arm, and right scapular region. He was diagnosed as primary benign generalized follicular mucinosis, and treated with isotretinoin. Almost complete remission was achieved in 4 months.