Hailey-Hailey disease (HHD; MIM 169600) is a rare chronic autosomal dominant skin disorder resulting from a mutation in APT2C1, which codes for a Ca2+/Mn2+ ATPase protein (hSPCA1) in the Golgi apparatus. HHD is clinically characterized by recurrent episodes of vesicles, erosions and erythematous plaques in the intertriginous areas. Triggering factors include trauma, friction, sweating, ultraviolet light exposure, pregnancy, and menstruation. Nail changes include the presence of longitudinal leuconychia. Histopathology shows areas of intraepidermal acantholysis causing the "dilapidated brick-wall" appearance. We present a case of HHD in a 33-year-old male. What was unique in our patient was the painful and therapy-resistant perianal involvement that led to significant morbidity. His mother also had HHD but other family members were unaffected. He had received several treatments earlier including topical antibiotics, antifungals, and corticosteroids without much improvement. We started him on carbon dioxide laser and presently he is under follow-up. We report this case because of its rarity.