Retiform hemangioendothelioma (RH) is extremely rare, and often involves the skin and subcutaneous tissues of distal extremities in young adults or children. Since its first description by Calonje in 1994, only a few primary multiple cases have been described in the literature. We present a case of unusual primary multiple RH on forearm and neck occurring in a 56 years old female patient. The patient presented with a slow-growing cutaneous plaque-like lesion on her left forearm, followed by another lesion at the site of neck for several years. In the skin biopsy examination, a diagnosis of angiosarcoma with cutaneous metastasis was made based on multiple lesions at different anatomic sites and vasoformative growth pattern with anastomosing channels under the microscopy. However, postoperative histological diagnosis of the lesion was primary multiple RH by thoroughly microscopical inspection and the presence of thin-walled interconnecting vascular channels arranged in a retiform pattern and absence of lymph node metastasis. Despite wide surgical excision with tumor-free margin, the tumor recurred at the neck 3 months after surgery. The second local excision was performed. The patient was on regular follow-up for 18 months without additional treatments. There was no sign of recurrence or lymph node metastasis. Due to its rarity, multiple RH might be erroneously interpreted as angiosarcoma with metastasis by those who were not familiar with this condition, or conduce to aggressive postoperative treatment. Therefore, thoroughly microscopical inspection and a long-term follow-up are helpful for surgeons and pathologists to obtain an accurate diagnosis and avoid clinical over-treatment.