Anaplastic lymphoma kinase (ALK) gene translocation occurs in 3%-5% of patients with non-small cell lung cancer (NSCLC), typically in younger patients. Crizotinib (tyrosine kinase inhibitor) has been considered as the standard of care for advanced ALK-positive lung cancer but it only gives a median progression-free survival of 7.7-11 months. A 41-year-old old man, former smoker, was diagnosed with NSCLC in the right lung with manifest pleural effusion. This case was complicated by a pleural empyema and because of a trapped lung, there was an indication for the construction of a thoracostomy. After confirmation of the ALK translocation, therapy with crizotinib was started. After 8 weeks, there was excellent response, and 6 months later, all lesions were undetectable on CT scan. There was also complete healing of the thoracostomy wound. This case describes a relatively young patient with a poor prognosis but with a remarkable and long-term response to crizotinib monotherapy.