Aim: To report a case of acute hydrops with a large stromal cleft, in a case of keratoconus, managed with compression sutures and perfluoropropane (C3F8) descemetopexy and subsequent management of corneal vascularization in that eye with combination therapy of laser and anti-vascular endothelial growth factor (anti-VEGF). Background: Acute hydrops in keratoconus occurs following a tear or rupture in the Descemet's membrane (DM), resulting in aqueous percolating into the stroma. The presence of large stromal clefts in acute hydrops has been described as a known risk factor for delayed resolution and persistent edema, which in turn can incite inflammation and vascularization and thereby adversely affect the chances of graft survival later. We describe such a case managed effectively with a combination of different treatment modalities. Case description: A 12-year-old boy with keratoconus presented with acute hydrops in his left eye. The acute hydrops was managed with compression sutures along with C3F8 descemetopexy. Subsequent development of persistent deep corneal vascularization and recurrence of inflammation after the resolution of hydrops was managed with a combination of argon laser photocoagulation and intrastromal anti-VEGF injections, resulting in complete regression of the deep vascularization and resolution of inflammation. Conclusion: In our case, a combination of management modalities to address severe acute hydrops and its subsequent complications resulted in complete regression of the superficial and deep vessels, thus heralding an optimal outcome for a future corneal graft. Clinical significance: There are no definite guidelines for the management of acute hydrops in keratoconus. A customized and judicious combination of various treatment modalities described in the literature for acute hydrops and its sequelae can result in an accepted outcome, which can pave the way for an optimum result with elective treatments like keratoplasty later on.