Malignant chondroid syringoma (MCS) is a rare tumor, of the sweat gland; only a few hundred such cases are reported in literature. A female presented with a subcutaneous swelling on the scalp with repeated recurrence and positive regional lymph nodes. Adequate planning for the treatment of this case was possible as preoperative diagnosis of MCS was documented by fine needle aspiration cytology (FNAC). The case was successfully managed with a multimodal approach, which included radical surgery and subsequent radiotherapy. The patient is symptom free after 25 months. The possibility of this type tumor should be entertained when multiple recurrences occur following adequate excision. FNAC has a definitive role in planning rational therapy.
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