Fine needle aspiration (FNA) was suggestive of a lymphoproliferative disorder, following which a wedge biopsy was performed from the superior edge of the lesion that revealed “a non-Hodgkin lymphoma, T-phenotype compatible with primary cutaneous anaplastic large cell lymphoma.” On IHC, the cells were diffusely positive for CD3 and CD30 and negative for CD20, ALK1, CD5, GATA3, CD10, BCL6, and CD56. Here, TNFRSF8 is linked to lymphoproliferative syndrome.