Sebaceous lymphadenoma is a rare benign neoplasm presenting predominantly in the parotid gland or in periparotid lymph nodes. It rarely transforms into a malignant tumor. We present a sebaceous lymphadenoma with an unusual clinical presentation with a malignant component, infiltration of lymph nodes, and lymphangiosis carcinomatosa. Sebaceous lymphadenoma usually presents as a well-circumscribed, painless, infraauricular mass. In our 87-years-old male patient, the clinical presentation was confluent reddish livid plaques at the left cheek and the left neck. In addition, cervical lymph node metastases were suspected. After skin biopsy, the tumor was excised by parotidectomy, skin excision, and neck dissection followed by a postoperative radiotherapy. To our knowledge, this is the sixth reported case of sebaceous lymphadenocarcinoma and the first case presenting with cervical lymph node metastases and dermatological symptoms revealing lymphangiosis carcinomatosa.