Primary headache associated with sexual activity (HAS) is classified within the fourth group of primary headaches in International Classification of Headache Disorders-3. Headache during sexual activity may develop due to life-threatening conditions, and early diagnosis is essential for adequate treatment. Excluding aetiologies for secondary headaches is pivotal in considering HAS. Understanding the pathophysiological mechanism of HAS will lead to new therapeutic targets and the utilisation of new treatment options. Awareness about HAS should be raised among specialists, and patients must be educated about their existing headaches and encouraged to seek treatment. In this chapter, the clinical features, diagnosis and management strategies of HAS will be discussed case-based.