Many reports have described the presence of alopecia areata (AA) associated with other autoimmune diseases, which support the autoimmune nature of AA. Additionally, AA has been reported in association with malignancy as a paraneoplastic symptom. In this report, we describe three patients with diffuse large B-cell lymphoma, alveolar soft part sarcoma, and cavernous sinus arteriovenous fistula with embolization treatment, respectively, who characteristically presented with rectangular-patterned occipital AA.