Collagenous spherulosis of the breast is a recently described lesion that was previously been seen only in a setting of benign, typical epitheliosis. We report that unusual case of a collagenous spherulosis of the breast in association with severely atypical epithelial hyperplasia in a 45-year-old woman. The patient presented an asymptomatic right lump and underwent a wide breast tissue resection. After a postoperative follow-up of twelve months, the patient remained well with no breast symptoms. Since atypical hyperplasia in the breast is considered predictive of relatively high risk of later carcinoma development, the finding of collagenous spherulosis with atypical hyperplasia should suggest a long-term follow-up. although at present the biological and prognostic significance of this association is unknown.