Background: A 15-year-old Caucasian male presented with 9-month history of a recurrent nodule on the nasal columella. The previous biopsy was reported as a neurofibroma. Methods: Frozen sections revealed a spindle cell neoplasm. Permanent section immunohistochemistry sections stained positive for vimentin and smooth muscle actin and negative for S100 and CD34, confirming the diagnosis of leiomyosarcoma. Results: The tumor was removed using Mohs micrographic surgery. Radiological work-up revealed no distant metastasis. There has been no local recurrence to date. Conclusions: Leiomyosarcoma is a difficult diagnosis to make clinically and requires histological confirmation. Re-biopsy of a "benign"growth may be necessary if clinicopathological correlation does not match with the clinical behavior of the tumor in question. Finally, Mohs micrographic surgery is a useful treatment modality for leiomyosarcomas, particularly those located in cosmetically-sensitive regions of the body such as the nose.
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