We present the first case of laryngeal intravascular lymphoma coexisting with in situ squamous cell carcinoma. The patient, a 53 years old man, presented with hoarseness starting a year ago and underwent laryngoscopy, which revealed two nodular lesions on his right vocal cord. The histological and immunohistochemical examination of the biopsy specimens established the diagnosis of in situ squamous cell carcinoma coexisting with intravascular lymphoma of T-cell origin. Taking in consideration all the available references, the larynx has not until now been reported as a primary site of involvement of intravascular (angiotropic) lymphomas, nor as a secondary location in the systematic course of this disease. Furthermore no cases have been reported in the literature, concerning the synchronous affection of the larynx by this lymphoma and in situ laryngeal carcinoma, or other type of neoplasm.