Adenoid cystic carcinoma (ACC) of the breast is a very rare neoplasm. It presents a triple-negative phenotype in most cases, but its prognosis is generally considered to be better than other breast cancers with the same immunohistochemical pattern. Due to its controversial features, no data are available in the literature regarding a consensus approach for ACC treatment, especially for subtypes with worse prognosis like solid basaloid ACC. We present for the first time a rare case of ACC with multifocal presentation treated with breast-conservative surgery and intraoperative electron radiotherapy, thus supporting this treatment of ACC in selected patients like young women affected by the solid basaloid variant who commonly present a worse prognosis. In this case, no local or systemic recurrence was detected after 30 months of follow-up.