The exact pathogenesis of hidradenitis suppurativa (HS) is still unproven. In the last few years numerous studies hypothesized that the disease is triggered by genetic and environmental factors. Follicular occlusion seems to be central to its development followed by follicular rupture, and immune responses finally leading to the development of clinical HS lesions. Data to date suggest that there is immune dysregulation in both the cutaneous and systemic immune system in patients with HS, but relative contributions from the innate and adaptive immune systems have not been fully elucidated. Furthermore, it can be further assumed that endocrine disorders related to follicular diseases can be implicated in the pathophysiologic pathways of HS. This review focuses on the key role of the immune system and endocrine disorders in the pathogenesis of HS.