A 17-year-old woman had an extensive nevus comedonicus affecting the left sides of the breast, trunk, axilla, and upper extremity. Her condition was complicated by episodes of re-current cyst formation, secondary infection, and atrophic scars. We performed various therapeutic approaches include topical keratolytic agents, manual extraction of comedones, excision of smaller lesions, and systemic antibiotics but the response was unsatisfactory. We tried systemic isotretinoin and minocycline and the pustulation of the lesions was reduced. Herein we report a rare case of linearly arranged extensive nevus comedonicus.