Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare cutaneous CD30 + T cell lymphoproliferative disorder. PCALCL usually presents as solitary and localized nodules and tumors, or may be multiple and ulcerated. Extracutaneous involvement may occur rarely. Systemic anaplastic large cell lymphoma with secondary cutaneous involvement has a similar clinical appearance to PCALCL, but differs in treatment and prognosis. Thus, systemic evaluation of patients considered as PCALCL is critical. An 82-year-old male patient presented with three progressive lesions on the scalp. The final diagnosis was PCALCL with extracutaneous involvement based upon the involvement of regional lymph nodes, liver and spleen, lack of systemic B symptoms and the results of histopathological and immunophenotypical studies. This case is presented to emphasize the necessity of systemic evaluation in patients considered as PCALCL and to remind how to differentiate PCALCL from systemic lymphomas.