Idiopathic granulomatous mastitis (IGM), is a rare entity of chronic inflammatory disorder of the breast of unknown etiology. Very few cases have been described so far, almost exclusively in women. Here we describe a case of IGM in a 53-year-old man presented with a right breast mass, progressively enlarging during the last 6 months. Due to the findings of clinical examination and CT-scan, the suspicion for a potentially malignant lesion was given and the decision for surgical resection was made. Microscopic analysis of the specimen showed non-caseating granulomas around mammary lobules, findings compatible with IGM.The patient is recurrence-free at 18-month follow-up. IGM is a rare benign inflammatory breast disease, usually seen in females of reproductive age. Establishing a diagnosis can be challenging for a surgeon and requires a high index of suspicion as most patients are initially misdiagnosed by their primary care physicians.Steroids and immunosuppressive drugs are considered as fundamental treatment modalities but they are correlated with increased rates of disease response and recurrence. On the contrary, surgical resection demonstrated significantly superior results compared to steroid-alone treatment in terms of recurrence and post-treatment recovery.