Introduction/Background Paget’s disease of the nipple is a rare condition that occurs in 1 to 4% of breast cancers. It corresponds to the infiltration of the epidermis of the nipple by adenocarcinoma-like cells. Clinically, it is manifested by eczematous eruptions on the nipple and areola. The association of Paget’s disease with a homolateral breast cancer is found in 82 to 100% of cases, hence the interest of our case

Methodology We report the case of an isolated Paget’s disease of the nipple in a 40 years old patient with no particular pathological history

Results Our patient was 40 years old with no particular pathological history and presented an eczematous rash on the left nipple. Radiologically, the breast ultrasound, mammography and MRI were normal, and a biopsy was done in favor of Paget’s disease.

Conclusion Paget’s disease of the nipple, although rare, should be considered in the presence of any eczematous lesion of the nipple that has not responded to medical treatment. Diagnostic uncertainty requires a histological comparison. Mammography and ultrasound of the breast, possibly with breast MRI, should be used to search for underlying breast cancer and guide surgical management. The prognosis of Paget’s disease of the breast is determined by the presence of a palpable mass and the invasiveness of the underlying cancer.

Disclosures Paget’s disease of the nipple, although rare, should be considered in the presence of any eczematous lesion of the nipple that has not responded to medical treatment. Diagnostic uncertainty requires a histological comparison. Mammography and ultrasound of the breast, possibly with breast MRI, should be used to search for underlying breast cancer and guide surgical management. The prognosis of Paget’s disease of the breast is determined by the presence of a palpable mass and the invasiveness of the underlying cancer.