BACKGROUND: Previous studies have reported cases of primary melanoma of the breast parenchyma (PMBP), but the pathogenesis of this disease remains poorly understood. We review the presentation and outcomes of reported cases and provide detailed pathological analysis of four additional cases. Furthermore, we discuss potential theories regarding the pathogenesis of this clinical presentation. RESULTS: We identified 29 published studies (n = 95 patients) and report four new cases (n = 99). Ninety-one (92 %) patients were female, with a median age of 50 years. Previous skin melanomas were reported by 56 % of patients, with the trunk being the most common location (32.7 %) followed by the upper extremities (20 %). The most common tumor location reported (n = 73) was the right (49 %) upper outer quadrant (56 %). The median time from skin melanoma diagnosis to the presence of a breast mass was 65 months (1-192). Nodal status at presentation was reported in n = 67 (68 %) patients. Of these, positive nodal metastases were seen in 40.3 %, while distant metastatic disease at presentation was reported in 30 % of patients. Surgery was performed in 66 %, being partial mastectomy (PM) the most common procedure in 82 %. Adjuvant therapy was described in 38 patients. The reported (n = 12) median survival was 11.5 (1-70) months. CONCLUSION: Melanomas identified in the breast parenchyma are likely the result of nodal or hematogenous spread from previously known or unknown melanomas, and should not be considered as PMBP. Management should be multidisciplinary, including surgical excision aimed at obtaining negative margins with lymphadenectomy of clinically positive nodes and neoadjuvant/adjuvant immunotherapy.