Mantle cell lymphoma is a rare neoplastic disease of the lymphatic system derived from mature B lymphocytes. It very commonly affects extranodal sites including the bone marrow, gastrointestinal tract and Waldeyer’s ring, but skin involvement is seen extremely rarely. The disease often has a recurrent and aggressive course. The diagnosis is usually established late, at an advanced stage of the disease. We present the case of a patient with skin lesions developing as the first manifestation of a haematological disease. After the appearance of symptoms the patient had several medical consultations before the final diagnosis was made at a dermatology outpatient clinic. Only few cases of cutaneous manifestations of mantle cell lymphoma have been described.