Introduction: A 51-year-old woman was diagnosed with a rare case of multi-focal ductal carcinoma in situ (DCIS) of the breast associated with Poland syndrome.
Clinical picture: Physical examination showed mild hypoplasia of the left breast with microcalcifications on mammogram. Intraoperatively, there was complete absence of the pectoralis major, which precluded the insertion of a breast implant.
Treatment and outcome: She underwent a skin-sparing mastectomy and autologous microsurgical free flap reconstruction with deep inferior epigastric perforator (DIEP) flap. Postoperative recovery was uneventful with no evidence of recurrence at 6 months.
Conclusion: This is the first reported case of DCIS of the breast with Poland syndrome. We performed a skin-sparing mastectomy with immediate DIEP flap recontruction. Patient recovered well postoperatively with no evidence of recurrence at 6 months.