According to the REAL classification, CD30+ anaplastic large cell lymphoma of the skin is a distinct clinicopathologic entity characterized by an indolent clinical course. Mycosis fungoides are predominantly small cells with cerebriform nuclei and propensity to infiltrate the epidermis or epidermotropism. CD30+ lymphoid cells are not generally observed in mycosis fungoides except in late or terminal stage. To distinguish between CD30 positive anaplastic large cell lymphoma and CD30 positive mycosis fungoides may be difficult. We describe a case of CD30 positive large cell lymphoma of skin that responded to bexarotene that is recently approved for refractory mycosis fungoides.