Literature is sparse regarding the management and long-term outcomes of breast cancer in patients with Ehlers-Danlos syndrome (EDS). Of the EDS subtypes, hypermobile Ehlers-Danlos Syndrome (hEDS) is associated with cardiovascular dysautonomia which manifests as spontaneous episodes of tachycardia and hypotension. Given this clinical autonomic system impact, hEDS is known to have significant intraoperative risk and postoperative complications. However, outcomes of hEDS patients have not been specifically studied in the field of breast cancer surgery. Here we present a case of a 62-year-old female with hEDS and node-positive invasive ductal breast carcinoma. Given the patient's medical history of hEDS, close attention was given to the patient's intraoperative vital signs and predisposition for poor wound healing. The patient underwent left Goldilocks mastectomy with left axillary lymph node dissection. Due to cardiac comorbidities, she was not a candidate for neoadjuvant or adjuvant chemotherapy. The patient tolerated adjuvant radiation and endocrine therapy without side effects, and has remained free of local, regional, and distant cancer recurrence following treatment. This case report highlights a literature gap in the surgical and radiation therapy management of breast cancer in patients with hEDS. Although breast surgery and radiation therapy in patients with invasive breast cancer and hEDS can be a safe management option, we discuss how perioperative complications must be cautiously navigated and how treatment must be tailored to individuals' specific hEDS variant to ensure optimal patient safety and positive long-term outcomes.