Invasive lobular carcinoma is the second most frequent histological type of breast cancer and its incidence is increasing. It has unique clinical, biological, and molecular features. Invasive lobular carcinoma is almost invariably positive for the oestrogen receptor and, when compared with invasive ductal carcinoma, it is typically of a lower grade. Even though invasive lobular carcinoma represents a distinct clinical entity, the same criteria used for invasive ductal carcinoma are currently applied to establish the need for primary or adjuvant systemic chemotherapy. We reviewed randomised trials of neoadjuvant and adjuvant chemotherapy and noted that insufficient evidence is available to support or withhold use of chemotherapy in patients with invasive lobular carcinoma. Thus, the benefit from systemic chemotherapy for individuals with this form of breast disease is unclear. Invasive lobular carcinoma deserves to be investigated separately in prospective clinical trials to define the best treatment and prevention strategies.