Anxiety can make it difficult for patients to manage their illness. Therefore, it is important to reduce their anxiety if possible. However, few studies have examined the efficacy of drugs in the treatment of anxiety in patients with cancer. Our case had failed to respond to benzodiazepines, and it was difficult to use a selective serotonin reuptake inhibitor (SSRI) as the next drug. This case report describes the effective use of quetiapine to treat anxiety. We report this rare case along with a literature review. Few studies have assessed the treatment of anxiety in patients with rare cancers. In our case, quetiapine effectively alleviated anxiety associated with cystic adenoid carcinoma. However, in clinical practice, it is possible that anxiety is treated without differentiating the effects of cancer status, e.g. life prognosis, treatment progress. In our patient, benzodiazepines had no effect on anxiety. Thus, different drugs may be required to treat anxiety associated with cancer. The present study demonstrated that quetiapine is a useful modality for the palliative care of patients with rare cancer and intractable anxiety. Quetiapine may be an effective alternative to benzodiazepines (BZ) and SSRIs for treating anxiety in patients with cancer. However, further investigation is needed to clarify the efficacy of treatments for anxiety associated with rare cancers.