PURPOSE: With the continuous development and progress of next-generation gene sequencing technology, many types of anaplastic lymphoma kinase (ALK) rearrangement have been discovered. However, in small cell lung cancer (SCLC), ALK rearrangement is extremely rare and there is no standard treatment protocol. By reviewing the literature, we summarized the previously reported cases of ALK-positive SCLC, and discussed the significance of molecular detection. METHOD: We report a rare patient with EML4-ALK fusion gene SCLC, a 41-year-old woman with no history of smoking or drinking, who was admitted to the hospital with chest tightness, dyspnea, and cough and sputum. Extensive SCLC (cT4N0M1) was diagnosed after relevant examination and pathological examination. The patient relapsed again six months after receiving first-line chemoradiotherapy. And the patient still developed disease progression (PD) after continued multi-line treatment including chemotherapy, immunotherapy, and anti-vascular therapy. ALK inhibitor is currently being taken orally, and significant clinical response has been achieved. Progression-free survival (PFS) was more than 8 months. RESULT: ALK rearrangement of SCLC is rare. The stage IV patient with ALK rearrangement benefit from ALK inhibitors after multiline therapy. CONCLUSION: For patients with ALK-positive SCLC, ALK inhibitors may be a reliable treatment option.