Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare adnexal neoplasm, believed to arise in a preexisting nevus sebaceus of Jadassohn (NSJ) through a multistep progression process. This hypothetical process involves an NSJ giving rise to syringocystadenoma papilliferum, which then presumably undergoes malignant transformation in rare circumstances to give rise to SCACP in situ, which finally progresses to an invasive SCACP. Of the 30 SCACP cases reported so far, none have documented the process from a birthmark to the final invasive lesion, with histological evidence of each step, in a single tumor. Here, the authors report just such a case. A 74-year-old man presented with a recently enlarging birthmark on the scalp. Excisional biopsy showed an invasive SCACP, in the background of SCACP in situ, syringocystadenoma papilliferum, and NSJ. Furthermore, this tumor showed a concurrent pigmented trichoblastoma and histological evidence of lymphovascular invasion, events that have not been documented with SCACP. Interestingly, all these component lesions were present on a single histological section of this solitary tumor. Regional lymph node metastasis, a rare occurrence in SCACP, was also present in this remarkable case. The authors discuss the implications of these findings in light of the review of relevant literature.