We present the case of a 37-year-old man with multiple pulmonary metastases of a primarily unknown primary tumour. Thorough revision of the medical history yielded that he had already passed three resections of a right palmar mass, which had been described as a benign tumour. Clinical examination showed a thickened scar with a suspicious palpable mass in the right hand. Excision of this scar and the tumour mass with histopathological examination now revealed a malignant acrospiroma. Resection of the pulmonary metastases histologically also confirmed a malignant acrospiroma. The following radical resection of the metacarpals II and III with the index and middle finger under the assumption of a wide compartment resection achieved tumour free margins and proved to be efficient with the patient being relapse free for 4 years from this operation. Although the prognosis of this tumour is generally unfavourable this particular case demonstrates the value of a radical surgical resection as the mainstay of treating such highly malignant sweat gland tumours of the hand.