Adenoid cystic carcinoma (ACC) of larynx is a rare disease, accounting for less than 1% of laryngeal malignancies, These tumors arise almost exclusively in the subglottic and supraglottic regions, while the proportion of glottic ACCs is small. We describe a case of a young man with a subglottis adenoid cystic carcinoma, The patient underwent laryngeal microscopic surgery and radiotherapy, Five years after radiotherapy, local recurrence and distant metastasis Clung and kidney) was detected and he underwent palliative chemotherapy and local treatment. He is still alive for seven years after the initial diagnosis. The treatment strategies for laryngeal ACC are still controversial due to the rarity of the condition. We suggest that multimodality approach may be helpful in deciding a treatment option, and thorough and consistent follow-up for recurrence is mandatory for these patients.