Atypical cutaneous lymphoid hyperplasia is a rare tumour of the head and neck which represents a lymphoproliferative continuum and mimics cutaneous lymphoma. Hereby reporting the case of a 40-year-old gentleman who presented with a swelling over dorsum of nose. On evaluation, Fine needle aspiration cytology was inconclusive with only inflammatory cells. Contrast Enhanced CT Nose and PNS suggested a possibility of a haemangioma. Patient underwent laser assisted excision of mass and frozen section showed sheets of basaloid cells suggestive of basal cell carcinoma. Wide local excision and primary paramedian forehead flap closure was done. Histopathology was suggestive of CD30 + atypical cutaneous lymphoid hyperplasia which has potential for malignant transformation into overt lymphoma. Patient underwent pedicle division of flap and is on regular follow up. In this case, aspiration cytology and frozen section reports were discordant with the histopathology, and thereby enlightens us of a rare differential diagnosis for head and neck tumours.
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